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We present a 42-year-old woman with a 1-year history of chronic rhinosinusitis, left facial palsy, bilateral sensorineural hearing loss, and bilateral necrotizing scleritis. Analytics revealed, elevated C-reactive protein and positive anti-proteinase 3. Tomography showed varicose and cystic bronchiectasis in both lungs and situs inversus totalis. It was concluded that the clinical assessment was compatible with Granulomatosis with polyangiitis in a patient with Kartagener’s Syndrome. She received immunosuppressive treatment with rituximab and high-dose corticosteroids. The evolution was partially favorable, because the patient already had irreversible organ damage at the time of diagnosis., Se presenta el caso de una mujer de 42 años con historia de 1 año de rinosinusitis crónica, parálisis facial izquierda, hipoacusia neurosensorial bilateral y escleritis necrotizante bilateral. En el laboratorio se encontró la proteína C reactiva elevada y anti-proteinasa3 positiva. En la tomografía se observaron bronquiectasias varicosas y quísticas en ambos pulmones y situs inversus totalis. Se concluyó que el cuadro clínico era compatible con granulomatosis con poliangitis y Síndrome de Kartagener. La paciente recibió tratamiento inmunosupresor con rituximab y corticoides a altas dosis. La evolución de la paciente fue parcialmente favorable debido a que presentaba daño orgánico irreversible al momento del diagnóstico.

Objective: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are inflammatory disorders with ANCA autoantibodies recognising either proteinase 3 (PR3-AAV) or myeloperoxidase (MPO-AAV). PR3-AAV and MPO-AAV have been associated with distinct loci in the human leucocyte antigen (HLA) region. While the association between MPO-AAV and HLA has been well characterised in East Asian populations where MPO-AAV is more common, studies in populations of European descent are limited. The aim of this study was to thoroughly characterise associations to the HLA region in Scandinavian patients with PR3-AAV as well as MPO-AAV. Methods: Genotypes of single-nucleotide polymorphisms (SNPs) located in the HLA region were extracted from a targeted exome-sequencing dataset comprising Scandinavian AAV cases and controls. Classical HLA alleles were called using xHLA. After quality control, association analyses were performed of a joint SNP/classical HLA allele dataset for cases with PR3-AAV (n=411) and MPO-AAV (n=162) versus controls (n=1595). Disease-associated genetic variants were analysed for association with organ involvement, age at diagnosis and relapse, respectively. Results: PR3-AAV was significantly associated with both HLA-DPB1*04:01 and rs1042335 at the HLA-DPB1 locus, also after stepwise conditional analysis. MPO-AAV was significantly associated with HLA-DRB1*04:04. Neither carriage of HLA-DPB1*04:01 alleles in PR3-AAV nor of HLA-DRB1*04:04 alleles in MPO-AAV were associated with organ involvement, age at diagnosis or relapse. Conclusions: The association to the HLA region was distinct in Scandinavian cases with MPO-AAV compared with cases of East Asian descent. In PR3-AAV, the two separate signals of association to the HLD-DPB1 region mediate potentially different functional effects.

Objective: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are inflammatory disorders with ANCA autoantibodies recognising either proteinase 3 (PR3-AAV) or myeloperoxidase (MPO-AAV). PR3-AAV and MPO-AAV have been associated with distinct loci in the human leucocyte antigen (HLA) region. While the association between MPO-AAV and HLA has been well characterised in East Asian populations where MPO-AAV is more common, studies in populations of European descent are limited. The aim of this study was to thoroughly characterise associations to the HLA region in Scandinavian patients with PR3-AAV as well as MPO-AAV. Methods: Genotypes of single-nucleotide polymorphisms (SNPs) located in the HLA region were extracted from a targeted exome-sequencing dataset comprising Scandinavian AAV cases and controls. Classical HLA alleles were called using xHLA. After quality control, association analyses were performed of a joint SNP/classical HLA allele dataset for cases with PR3-AAV (n=411) and MPO-AAV (n=162) versus controls (n=1595). Disease-associated genetic variants were analysed for association with organ involvement, age at diagnosis and relapse, respectively. Results: PR3-AAV was significantly associated with both HLA-DPB1*04:01 and rs1042335 at the HLA-DPB1 locus, also after stepwise conditional analysis. MPO-AAV was significantly associated with HLA-DRB1*04:04. Neither carriage of HLA-DPB1*04:01 alleles in PR3-AAV nor of HLA-DRB1*04:04 alleles in MPO-AAV were associated with organ involvement, age at diagnosis or relapse. Conclusions: The association to the HLA region was distinct in Scandinavian cases with MPO-AAV compared with cases of East Asian descent. In PR3-AAV, the two separate signals of association to the HLD-DPB1 region mediate potentially different functional effects.

Objective: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are inflammatory disorders with ANCA autoantibodies recognising either proteinase 3 (PR3-AAV) or myeloperoxidase (MPO-AAV). PR3-AAV and MPO-AAV have been associated with distinct loci in the human leucocyte antigen (HLA) region. While the association between MPO-AAV and HLA has been well characterised in East Asian populations where MPO-AAV is more common, studies in populations of European descent are limited. The aim of this study was to thoroughly characterise associations to the HLA region in Scandinavian patients with PR3-AAV as well as MPO-AAV. Methods: Genotypes of single-nucleotide polymorphisms (SNPs) located in the HLA region were extracted from a targeted exome-sequencing dataset comprising Scandinavian AAV cases and controls. Classical HLA alleles were called using xHLA. After quality control, association analyses were performed of a joint SNP/classical HLA allele dataset for cases with PR3-AAV (n=411) and MPO-AAV (n=162) versus controls (n=1595). Disease-associated genetic variants were analysed for association with organ involvement, age at diagnosis and relapse, respectively. Results: PR3-AAV was significantly associated with both HLA-DPB1*04:01 and rs1042335 at the HLA-DPB1 locus, also after stepwise conditional analysis. MPO-AAV was significantly associated with HLA-DRB1*04:04. Neither carriage of HLA-DPB1*04:01 alleles in PR3-AAV nor of HLA-DRB1*04:04 alleles in MPO-AAV were associated with organ involvement, age at diagnosis or relapse. Conclusions: The association to the HLA region was distinct in Scandinavian cases with MPO-AAV compared with cases of East Asian descent. In PR3-AAV, the two separate signals of association to the HLD-DPB1 region mediate potentially different functional effects.

Objective: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are inflammatory disorders with ANCA autoantibodies recognising either proteinase 3 (PR3-AAV) or myeloperoxidase (MPO-AAV). PR3-AAV and MPO-AAV have been associated with distinct loci in the human leucocyte antigen (HLA) region. While the association between MPO-AAV and HLA has been well characterised in East Asian populations where MPO-AAV is more common, studies in populations of European descent are limited. The aim of this study was to thoroughly characterise associations to the HLA region in Scandinavian patients with PR3-AAV as well as MPO-AAV. Methods: Genotypes of single-nucleotide polymorphisms (SNPs) located in the HLA region were extracted from a targeted exome-sequencing dataset comprising Scandinavian AAV cases and controls. Classical HLA alleles were called using xHLA. After quality control, association analyses were performed of a joint SNP/classical HLA allele dataset for cases with PR3-AAV (n=411) and MPO-AAV (n=162) versus controls (n=1595). Disease-associated genetic variants were analysed for association with organ involvement, age at diagnosis and relapse, respectively. Results: PR3-AAV was significantly associated with both HLA-DPB1*04:01 and rs1042335 at the HLA-DPB1 locus, also after stepwise conditional analysis. MPO-AAV was significantly associated with HLA-DRB1*04:04. Neither carriage of HLA-DPB1*04:01 alleles in PR3-AAV nor of HLA-DRB1*04:04 alleles in MPO-AAV were associated with organ involvement, age at diagnosis or relapse. Conclusions: The association to the HLA region was distinct in Scandinavian cases with MPO-AAV compared with cases of East Asian descent. In PR3-AAV, the two separate signals of association to the HLD-DPB1 region mediate potentially different functional effects.

Objective: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are inflammatory disorders with ANCA autoantibodies recognising either proteinase 3 (PR3-AAV) or myeloperoxidase (MPO-AAV). PR3-AAV and MPO-AAV have been associated with distinct loci in the human leucocyte antigen (HLA) region. While the association between MPO-AAV and HLA has been well characterised in East Asian populations where MPO-AAV is more common, studies in populations of European descent are limited. The aim of this study was to thoroughly characterise associations to the HLA region in Scandinavian patients with PR3-AAV as well as MPO-AAV. Methods: Genotypes of single-nucleotide polymorphisms (SNPs) located in the HLA region were extracted from a targeted exome-sequencing dataset comprising Scandinavian AAV cases and controls. Classical HLA alleles were called using xHLA. After quality control, association analyses were performed of a joint SNP/classical HLA allele dataset for cases with PR3-AAV (n=411) and MPO-AAV (n=162) versus controls (n=1595). Disease-associated genetic variants were analysed for association with organ involvement, age at diagnosis and relapse, respectively. Results: PR3-AAV was significantly associated with both HLA-DPB1*04:01 and rs1042335 at the HLA-DPB1 locus, also after stepwise conditional analysis. MPO-AAV was significantly associated with HLA-DRB1*04:04. Neither carriage of HLA-DPB1*04:01 alleles in PR3-AAV nor of HLA-DRB1*04:04 alleles in MPO-AAV were associated with organ involvement, age at diagnosis or relapse. Conclusions: The association to the HLA region was distinct in Scandinavian cases with MPO-AAV compared with cases of East Asian descent. In PR3-AAV, the two separate signals of association to the HLD-DPB1 region mediate potentially different functional effects.

Objectives To examine long-term cardiovascular outcomes and temporal trends among patients with ANCA-associated vasculitis (AAV) using Danish nationwide registries. Methods Using a cohort design, we examined patients with granulomatosis with polyangiitis (ICD-10: DM31.3) and microscopic polyangiitis (ICD-10: DM3.17) in Denmark from 1996-2018. Hazard ratios (HRs) of cardiovascular outcomes were compared between patients with AAV and age and gender-matched controls. Counterfactual G-estimation of HRs was performed to estimate 5-year absolute risks. Temporal trends were obtained by grouping cohorts into evenly distributed tertiles according to inclusion year. Results A total of 2306 patients with AAV (median age: 62.9yrs, 52.6% male) were matched with 6918 controls. Median follow-up was 9.5yrs. Patients with AAV had a higher rate of ischaemic heart disease [HR 1.86 (1.62-2.15)], myocardial infarction [HR 1.62 (1.26-2.09)], coronary angiogram [HR 1.64 (1.37-1.96)], percutaneous coronary intervention [HR 1.56 (1.17-2.07)] and ventricular arrhythmias/implantable-cardioverter-defibrillator (ICD)-implantations [HR 2.04 (1.16-3.57)]. Similarly, an increased rate of heart failure [HR 2.12 (1.77-2.54)], deep vein thrombosis [HR 3.13 (2.43-4.05)], pulmonary embolism [HR 4.04 (3.07-5.32)], atrial fibrillation [HR 2.08 (1.82-2.39)], ischaemic stroke [HR 1.58 (1.31-1.90)] and in-hospital cardiac arrest [HR 2.27 (1.49-3.48)] was observed. The 5-year risk of all outcomes were significantly higher (excluding ventricular arrhythmia/ICD-implantations). For temporal trends among patients with AAV, a decreased 3-year risk of cardiovascular mortality was observed over time. Conclusions Patients with AAV are at increased risk of heart failure, atrial-/ventricular arrhythmias, venous thrombotic events, ischaemic stroke and myocardial infarction. Furthermore, patients with AAV were more frequently examined with coronary procedures and underwent more coronary revascularizations. No tempora

OBJECTIVE: To study the prevalence, risk and clinical associations of hypothyroidism among several forms of vasculitis. METHODS: Patients with GCA, Takayasus arteritis (TAK), PAN and the three forms of ANCA-associated vasculitis [AAV; granulomatosis with polyangiitis (GPA), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (EGPA)] enrolled in a prospective, multicentre, longitudinal study were included. RESULTS: The study included data on 2085 patients [63% female, 90% White] with a mean age of 54.6 years (s.d. 17.2). Diagnoses were GCA (20%), TAK (11%), PAN (5%), GPA (42%), microscopic polyangiitis (8%) and EGPA (14%). Hypothyroidism was present in 217 patients (10%) (83% female), with a mean age 59.8 years (s.d. 14.5). Age- and sex-adjusted risk of hypothyroidism was GCA, odds ratio (OR) 0.61 (95% CI 0.41, 0.90); TAK, OR 0.57 (95% CI 0.31, 1.03); PAN, OR 0.59 (95% CI 0.25, 1.38); GPA, OR 1.51 (95% CI 1.12, 2.05); microscopic polyangiitis, OR 1.81 (95% CI 1.18, 2.80) and EGPA, OR 0.82 (95% CI 0.52, 1.30). Among patients with AAV, age- and sex-adjusted risk of hypothyroidism was higher with positive MPO-ANCA [OR 1.89 (95% CI 1.39, 2.76)]. The clinical manifestations of vasculitis were similar in patients with and without hypothyroidism, except transient ischaemic attacks, which were more frequently observed in patients with GCA and hypothyroidism (12% vs 2%; P = 0.001). CONCLUSIONS: Differences in the risk of hypothyroidism among vasculitides may be due to genetic susceptibilities or immune responses. This study confirms an association of hypothyroidism with MPO-ANCA.

Objective. To examine the long-term rates of heart failure (HF) and other adverse cardiovascular (CV) outcomes in a nationwide cohort of patients diagnosed with granulomatosis with polyangiitis (GPA) compared with the general population. Methods. Using Danish nationwide registries, patients with newly diagnosed GPA were identified and matched 1:4 by age, sex, and comorbidities with subjects from the general population. Outcomes were compared using Cox regression. Due to violation of the proportional hazard assumption, landmark analyses for the first year and from 1 year were performed. Results. Of the 1923 patients with GPA, 1781 patients (median age 59 yrs, 47.9% men) were matched with 7124 subjects from the general population. The median follow-up was 6.4 years. The absolute 10-year risk of HF was 6.8% (95% CI 5.5–8.2%) for patients with GPA and 5.9% (5.3–6.6%) for the general population. During the first year after diagnosis, GPA was associated with a significantly higher rate of HF (hazard ratio [HR] 3.60, 95% CI, 2.28–5.67) and other adverse outcomes, including atrial fibrillation/flutter (HR 6.50, 95% CI 4.43–9.55) and ischemic stroke (HR 3.24, 95% CI 1.92–5.48), compared with the general population. After the first year, GPA was not associated with higher rates of HF or other CV outcomes compared with the general population, except atrial fibrillation/flutter (HR 1.38, 95% CI 1.12-1.70). Conclusion. During the first year after diagnosis, the rates of HF and other CV outcomes were higher in patients with GPA compared with the general population. However, after the first year, the rates of HF and other CV outcomes, except atrial fibrillation/flutter, were similar to those in the general population.

Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) are systemic necrotizing vasculitides associated with significant morbidity and mortality. Given the immunosuppression used to manage these conditions, it is important for clinicians to recognize complications, especially infectious ones, which may arise during treatment. Kaposi sarcoma (KS) is a lymphoangioproliferative neoplasm caused by human herpes virus 8 (HHV-8). Its cutaneous manifestations can mimic vasculitis. We describe a 77-year-old man with microscopic polyangiitis with pulmonary-renal syndrome treated with prednisone and intravenous cyclophosphamide who developed KS (HHV-8 positive) after 2 months of treatment. Cyclophosphamide was discontinued and prednisone gradually lowered with improvement and clinical stabilization of KS lesions. This comprehensive review includes all published cases of KS in patients with AAV, with a goal to summarize potential risk factors including the clinical characteristics of vasculitis, treatment and outcomes of patients with this rare complication of immunosuppressive therapy. We also expanded our literature review to KS in other forms of systemic vasculitis. Our case-based review emphasizes the importance of considering infectious complications of immunosuppressive therapy, especially glucocorticoids, and highlights the rare association of KS in systemic vasculitis.

Pyoderma gangrenosum (PG) is an inflammatory, ulcerative condition that is characterized by painful ulcers that commonly present on the lower extremities. Up to half of PG cases are associated with underlying systemic disease, including inflammatory bowel disease, various autoimmune conditions, and malignancy. Another well-known association is the manifestation of PG with recreational cocaine use, especially cocaine contaminated with the adulterant agent levamisole. Once utilized for its immunomodulatory capabilities, levamisole was withdrawn from the market in 2002. It has since been repurposed to potentiate the amphetamine-like effects and duration of cocaine and has reduced preparation cost. We present a 52-year-old woman with chronic maxillary sinusitis and cocaine use disorder presenting with a two-week history of painful ulcers on bilateral lower extremities, each with a purulent base and undermined, violaceous borders. Urine toxicology was positive for cocaine and serologic studies were positive for cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) and lupus anticoagulant. Underlying conditions, especially that of granulomatosis with polyangiitis, were considered and ultimately ruled out. The patient's lesions exhibited a marked response with a short course of oral corticosteroids, typical of PG associated with levamisole. This case highlights the crucial role that drug abstinence plays in the prevention of recurrence.

Pyoderma gangrenosum (PG) is an inflammatory, ulcerative condition that is characterized by painful ulcers that commonly present on the lower extremities. Up to half of PG cases are associated with underlying systemic disease, including inflammatory bowel disease, various autoimmune conditions, and malignancy. Another well-known association is the manifestation of PG with recreational cocaine use, especially cocaine contaminated with the adulterant agent levamisole. Once utilized for its immunomodulatory capabilities, levamisole was withdrawn from the market in 2002. It has since been repurposed to potentiate the amphetamine-like effects and duration of cocaine and has reduced preparation cost. We present a 52-year-old woman with chronic maxillary sinusitis and cocaine use disorder presenting with a two-week history of painful ulcers on bilateral lower extremities, each with a purulent base and undermined, violaceous borders. Urine toxicology was positive for cocaine and serologic studies were positive for cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) and lupus anticoagulant. Underlying conditions, especially that of granulomatosis with polyangiitis, were considered and ultimately ruled out. The patient's lesions exhibited a marked response with a short course of oral corticosteroids, typical of PG associated with levamisole. This case highlights the crucial role that drug abstinence plays in the prevention of recurrence.

Rationale: Bronchial involvement alone is a rare initial manifestation of granulomatosis with polyangiitis (GPA). Herein, we report a case of refractory GPA with obstructive pneumonia caused by bronchial involvement. Patient concerns: A 65-year-old man complained of a 2-week cough and fever. Diagnoses: Considering the presence of opacities and multiple consolidations in both lungs due to obstruction or stenosis on the bronchus, which did not respond to antibiotics, and proteinase-3-antineutrophil cytoplasmic autoantibody positivity, he was diagnosed with GPA. Positron emission tomography- computed tomography scan revealed no abnormal findings in the upper respiratory tract. Interventions: He was treated with prednisolone (PSL, 50 mg/d) and intravenous cyclophosphamide. Outcomes: His general and respiratory symptoms improved. However, 8 weeks after PSL treatment at 20 mg/d, he developed a relapse of vasculitis along with sinusitis and hypertrophic pachymeningitis. Hence, PSL treatment was resumed to 50 mg/d, and weekly administration of rituximab was initiated. Consequently, the symptoms gradually mitigated. Lessons: GPA with bronchial involvement is often intractable and requires careful follow-up, which should include upper respiratory tract and hypertrophic pachymeningitis assessment.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

Granulomatosis with polyangiitis (GPA) or also known as Wegener's Granolomatosis, is a type of systemic vasculitis that affects small vessels, most commonly affecting the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, images, and histology. Ninety percent are ANCA (anti-neutrophil cytoplasmic antibodies) positive. Histolo-gy demonstrates granulomatous inflammation, necrosis, and vasculitis, while imaging studies are vital for initial evaluation and follow-up. Thus, computed tomography (CT) is the method of choice for evaluating the upper airway and lungs, due to its high sensitivity to detect abnormalities of the paranasal sinuses, the bronchial tree, and the lungs. In addition to this, magnetic resonance imaging is indicated for the evaluation of the central nervous system and the heart, however, PET / CT positron emission tomography has high sensitivity for thoracic and abdominal diseases, it is useful to detect lesions that are not seen with other imaging techniques and for monitoring. Renal involvement, very common in GPA, is usually undetectable on imaging, but can be seen on PET / CT in cases of marked glomerulonephritis. However, plain radiographs should not be used to assess GPA due to their poor diagnostic performance. The patients are treated with corticosteroids and immunosuppressants and should be followed long-term, since relapses are generally frequent., Granulomatose com poliangiíte (GPA) ou também conhecida como Granolomatose de Wegener, é um tipo de vasculite sistêmica que afeta pequenos vasos, afetando mais comumente o trato respiratório e os rins. O diagnóstico é baseado em critérios clínicos, exames laboratoriais, imagens e histologia. Noventa por cento são ANCA (anticorpos citoplasmáticos anti-neutrófilos) positivos. A histologia demonstra inflamação granulomatosa, necrose e vasculite, enquanto os estudos de imagem são vitais para avaliação inicial e acompanhamento. Assim, a tomografia computadorizada (TC) é o método de escolha para avaliação das vias aéreas superiores e dos pulmões, devido à sua alta sensibilidade para detectar anormali-dades dos seios paranasais, árvore brônquica e pulmões. Além disso, a ressonância magnética é indicada para avaliação do sistema nervoso central e do coração, porém a tomografia por emissão de pósitrons PET / CT tem alta sensibilidade para doenças torácicas e abdominais, é útil para detectar lesões que não são observadas com outras técnicas de imagem e para monitoramento. O envolvimento renal, muito comum no GPA, geralmente é indetectável na imagem, mas pode ser observado na PET / TC em casos de glomerulonefrite acentuada. No entanto, radiografias simples não devem ser usadas para avaliar GPA devido ao seu baixo desempenho diagnóstico. Os pacientes são tratados com corticosteroides e imu-nossupressores e devem ser acompanhados por longo prazo, pois as recidivas geralmente são frequentes., La granulomatosis con poliangítis (GPA) o también conocida como Granolomatosis de Wegener, es un tipo de vasculitis sistémica que afecta los vasos pequeños, más comúnmente afectando el tracto respiratorio y los riñones. El diagnóstico se basa en criterios clínicos, pruebas de laboratorio, imágenes e histología. El noventa por ciento son ANCA (anticuerpos anticitoplasma de neutrofílico) positivos. La histología demuestra inflamación granulomatosa, necrosis y vasculitis, mientras que, los estudios de imágenes son vitales para la evaluación inicial y el seguimiento. De tal manera, la tomografía computarizada (TC) es el método de elección para la evaluación de la vía aérea superior y los pulmones, debido a su alta sensibilidad para detectar anomalías de los senos paranasales, el árbol bronquial y los pulmones. Aunado a ello, la resonancia magnética está indicada para la evaluación del sistema nervioso central y el corazón, sin embargo, la tomografía por emisión de positrones PET/TC tiene alta sensibilidad para enfermedades torácicas y abdominales, es útil para detectar lesiones que no se ven con otras técnicas de imagen y para el seguimiento. La afectación renal, muy frecuente en la GPA, suele ser indetectable en las imágenes, pero puede verse en la PET/TC en casos de glomerulonefritis marcada. No obstante, las radiografías simples no deben usarse para evaluar el GPA debido a su bajo rendimiento diagnóstico. Los pacientes son tratados con corticosteroides e inmunosupresores y deben llevar un seguimiento a largo plazo, ya que, generalmente las recaídas son frecuentes.

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Stenting is necessary when vascular patency is not adequately achieved with angioplasty alone. Common causes of pseudoaneurysms are iatrogenic and traumatic. We describe a case of a perforated left subclavian vein Gianturco stent causing a pseudoaneurysm of the left common carotid artery in a patient with granulomatosis with polyangiitis, detected 8 years after stent insertion.

ObjectiveTo describe a case series of previously undiagnosed granulomatosis with polyangiitis (GPA) patients who presented primarily with otological manifestations.MethodWe report a series of patients visited at a neurotology clinic who were eventually diagnosed with GPA based on their otologic complaints and had no prior knowledge of having this condition.ResultsIn this series, 10 (91%) patients presented with hearing loss (HL), more than half of which were bilateral (60%). Upon audiometric examination, all but 1 patient had mixed, conductive, or sensorineural HL. All patients presented with eustachian tube dysfunction (ETD), otitis media with effusion (OME), or both. Nasal endoscopy showed intranasal pathology in 3 (27%) patients. Otologic symptoms were improved in all patients after treatment with an average of 4 in-office follow-up appointments.ConclusionGPA should be included in the differential diagnosis of adults with unexplained mixed hearing loss, new onset serous effusion, or acute otitis media in the absence of a previous history of ETD. Laboratory tests (ie, anti-neutrophil cytoplasmic autoantibody, erythrocyte sedimentation rate, and C-reactive protein) along with a urinalysis can aid in screening these patients. In cases in which the index of suspicion is high, repeated testing could reduce the risk of false negative findings.

ObjectiveTo describe a case series of previously undiagnosed granulomatosis with polyangiitis (GPA) patients who presented primarily with otological manifestations.MethodWe report a series of patients visited at a neurotology clinic who were eventually diagnosed with GPA based on their otologic complaints and had no prior knowledge of having this condition.ResultsIn this series, 10 (91%) patients presented with hearing loss (HL), more than half of which were bilateral (60%). Upon audiometric examination, all but 1 patient had mixed, conductive, or sensorineural HL. All patients presented with eustachian tube dysfunction (ETD), otitis media with effusion (OME), or both. Nasal endoscopy showed intranasal pathology in 3 (27%) patients. Otologic symptoms were improved in all patients after treatment with an average of 4 in-office follow-up appointments.ConclusionGPA should be included in the differential diagnosis of adults with unexplained mixed hearing loss, new onset serous effusion, or acute otitis media in the absence of a previous history of ETD. Laboratory tests (ie, anti-neutrophil cytoplasmic autoantibody, erythrocyte sedimentation rate, and C-reactive protein) along with a urinalysis can aid in screening these patients. In cases in which the index of suspicion is high, repeated testing could reduce the risk of false negative findings.

Granulomatosis with polyangiitis (GPA) is one of three described anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). Early diagnosis and treatment of GPA is paramount, as it may help prevent irreversible end-organ damage, especially renal and pulmonary failure. A 72-year-old male with a past medical history of lung adenocarcinoma in remission, chronic sinusitis status-post multiple sinus surgeries, and coronary artery disease presented with shortness of breath, dark urine, and asymmetric polyarthralgias. He had an acute kidney injury, leukocytosis, with urinalysis demonstrating pyuria and hematuria, without casts. Chest imaging showed cavitary nodular opacities in addition to interval increase of existing nodules compared to the most recent scan one month prior. His acute kidney injury progressed to renal failure requiring hemodialysis, and he developed an inflammatory polyarthritis. GPA was suspected clinically so he was started on high-dose intravenous corticosteroids, and subsequently plasmapheresis and rituximab. Serology returned with highly positive proteinase-3 antibodies, and cytoplasmic ANCA positivity on immunofluorescence. Renal biopsy demonstrated severely active pauci-immune glomerulonephritis. Several months after discharge, the patient passed away from gram positive bacteremia. This patient's recurrent sinusitis, pulmonary nodules, and subsequent renal failure were highly suggestive of GPA. A biopsy is recommended to confirm the diagnosis of GPA, but treatment should not be delayed if there is a high index of suspicion for the disease. Induction therapy with corticosteroids combined with rituximab or cyclophosphamide has significantly decreased the mortality of patients with GPA. Patients with GPA often have preceding history of nasopharyngeal and upper airway disease, and can present with fluctuating pulmonary infiltrates. Early recognition and trea

Objective. The objective of this study was to identify risk factors for a relapse at the time of an increase in antineutrophil cytoplasmic antibodies (ANCA) in patients with renal ANCA-associated vasculitis (AAV).Methods. All patients between January 2000 and November 2011 with renal AAV having an ANCA rise during remission were included. Differences in time to relapse since the ANCA rise were assessed using a Cox regression model. The level of 25-hydroxy Vitamin D (25(OH) D) was assessed at the ANCA rise and at a subsequent relapse or time-matched during remission.Results. Sixty patients had an ANCA rise, of whom 36 patients relapsed. Three risk factors were associated with a relapse at the time of the ANCA increase: previous disease activity not treated with cyclophosphamide or rituximab (HR 3.48, 95% CI 1.60-7.59), an ANCA rise during the fall season (HR 4.37, 95% CI 1.60-11.90), and an extended ANCA rise (HR 3.57, 95% CI 1.50-8.48). Levels of 25(OH) D significantly decreased during followup in relapsing patients, but not in patients who remained in remission (difference -6.3 +/- 14.4, p = 0.017 vs 2.7 +/- 16.3, p = 0.430).Conclusion. ANCA rises occurring during the fall season are more frequently followed by a relapse than ANCA rises occurring during other seasons. Although it is tempting to speculate that decreasing Vitamin D levels following the ANCA rise can be held responsible for the subsequent relapse, this remains to be determined.

Hintergrund. Vielversprechende Biomarker konnten durch diverse Arbeiten, welche PatientInnen mit Anti-Neutrophil zytoplasmatische Antikörper (ANCA)-assoziierten Vaskulitis untersuchten, identifiziert werden. Das Ziel unserer Studie war die Validierung dieser Marker bei PatientInnen mit Granulomatose mit Polyangiitis oder mikroskopischer Polyangiitis und die Erstellung eines Biomarker-Panels, welches aktive PatientInnen von inaktive unterscheidet. Methoden. Ein systematischer Review wurde durchgeführt, welcher 161 Marker fand. Diese wurden nach deren quantitativen Expression bei aktiver und inaktiver Erkrankung geordnet. ELISA-kits wurden zur Validierung der Resultate in unserer Querschnittspopulation mit ANCA-assoziierter Vaskulitis und Nierenbeteiligung verwendet. Die Krankheitsaktivität wurde als Birmingham Vasculitis Score version 3 (BVAS v3) gemessen und eine aktive Erkrankung wurde definiert als BVAS v3 1 und Remission als BVAS v3 von 0. Die statistische Analyse wurde mittels SPSS Version 21 und dem Salford Predictive Modeler 7.0 durchgeführt, um Biomarker-Panels zu erstellen. Ergebnisse. Unser Review hat eine erhöhte Expression von sC5bC9, C3a, C5a und monocyte chemotactic protein (MCP)-1 im Harn nachgewiesen. Zudem zeigten granulocyte macrophage colony-stimulating factor, C-reaktives Protein (CRP), soluble fms-like tyrosine kinase-1, Interleukin-17A (IL-17A), C5a, Hyaluronsäure, C3a und Interleukin-18 binding protein (IL-18 bp) im Blut eine deutliche Erhöhung während aktiver Erkrankung. In unserer Querschnittsanalyse konnten wir eine signifikante Hochregulierung von CRP, C5a, C3a, IL-18 bp im Blut sowie C5a und MCP-1 im Harn während aktiver Krankheit nachweisen (p-Wert von <0.05). Die Erstellung eines Biomarker-Panels zeigte die beste Diskriminierung von CRP im Blut sowie MCP-1 im Harn, wobei die Sensitivität und Spezifität jeweils 76% betrugen (AUC von 0.89). Schlussfolgerungen. Wir haben im Rahmen eines Review Biomarker identifiziert, die teils in unserer Q, Background. Studies in anti-neutrophil cytoplasm antibody (ANCA)associated vasculitis (AAV) have revealed promising biomarkers. The aim of our study was to validate the most encouraging markers of granulomatosis with polyangiitis and microscopic polyangiitis identified by a literature search and to create biomarker panels distinguishing active from inactive disease. Methods. A systematic literature review was performed and we identified 161 marker molecules that were ranked by their quantitative differential expression between active and inactive disease. Enzyme-linked immunosorbent assays were used to validate the results in a cross-sectional cohort of patients with renal involvement. Active vasculitis as assessed by the Birmingham Vasculitis Score version 3 (BVAS v3) was defined as BVAS v3 1 and inactive disease as BVAS v3 = 0. Statistical analysis was performed with SPSS version 21 and the Salford Predictive Modeler 7.0 was used to generate a predictive biomarker panel. Results. The review indicated abundant expression of sC5bC9, C3a, C5a and monocyte chemotactic protein (MCP)-1 in urine, whereas granulocyte macrophage colony- stimulating factor, C-reactive protein (CRP), soluble fms-like tyrosine kinase-1, interleukin-17A (IL-17A), C5a, hyaluronan, C3a and interleukin-18 binding protein (IL-18 bp) were identified to be highly diverse in active and inactive disease in blood samples. Our cross-sectional analysis revealed significant up-regulation of CRP, C5a, C3a, IL-18 bp in blood and C5a and MCP-1 in urine samples during active AAV (all P < 0.05). Creation of a biomarker panel comprising CRP and urinary MCP-1 yielded a sensitivity and specificity of 76% (area under the curve 0.89). Conclusions. We identified promising biomarkers in a literature-based review that were in part corroborated as has been shown for CRP, C3a, C5a, IL-18 bp in blood and MCP-1 and C5a in urine samples. Moreover, we propose a biomarker panel comprising CRP and urinary MCP-1 in patients wi, eingereicht von Dr.med.univ. Andreas Kronbichler, Zusammenfassung in deutscher Sprache, Medizinische Universität Innsbruck, Dissertation, 2017, OeBB, (VLID)1926488

Objective. The objective of this study was to identify risk factors for a relapse at the time of an increase in antineutrophil cytoplasmic antibodies (ANCA) in patients with renal ANCA-associated vasculitis (AAV).Methods. All patients between January 2000 and November 2011 with renal AAV having an ANCA rise during remission were included. Differences in time to relapse since the ANCA rise were assessed using a Cox regression model. The level of 25-hydroxy Vitamin D (25(OH) D) was assessed at the ANCA rise and at a subsequent relapse or time-matched during remission.Results. Sixty patients had an ANCA rise, of whom 36 patients relapsed. Three risk factors were associated with a relapse at the time of the ANCA increase: previous disease activity not treated with cyclophosphamide or rituximab (HR 3.48, 95% CI 1.60-7.59), an ANCA rise during the fall season (HR 4.37, 95% CI 1.60-11.90), and an extended ANCA rise (HR 3.57, 95% CI 1.50-8.48). Levels of 25(OH) D significantly decreased during followup in relapsing patients, but not in patients who remained in remission (difference -6.3 +/- 14.4, p = 0.017 vs 2.7 +/- 16.3, p = 0.430).Conclusion. ANCA rises occurring during the fall season are more frequently followed by a relapse than ANCA rises occurring during other seasons. Although it is tempting to speculate that decreasing Vitamin D levels following the ANCA rise can be held responsible for the subsequent relapse, this remains to be determined.

Objective: Determine the frequency of granulomatosis with polyangiitis (GPA) associated with non-identified ANCA (non-MPO, non-PR3 ANCA) and secondarily compare their clinic with GPA associated with MPO-positive or PR3-positive ANCA. Methods: In a monocentric retrospective observational study, clinical data of 398 patients with non-identified ANCA (titer of ANCA at least 1/80 by immunofluorescence on ethanol fixed PMN) was gathered over a period of 6 years. GPA patients from this population were compared with GPA patients with identified ANCA on the basis of clinical, biological, immunological and histological features. Results: The most common diseases associated with non-identified ANCA were inflammatory bowel diseases accounting for 17% of diseases. GPA accounted for only 1.8% of cases. There were no significant differences in terms of clinical and histological characteristics between GPA with non-identified ANCA and GPA with identified ANCA, but significantly higher CRP levels were observed in GPA patients with identified ANCA (p = 0.005). Localized disease (ear, nose and throat and/or lung involvement without any other systemic involvement) was more frequent in the group of GPA with nonidentified ANCA (p = 0.047) as compared to GPA with identified ANCA. This explains that the former group of patients was less frequently treated by cyclophosphamide than the latter (p = 0.016). Conclusion: GPA with non-MPO, non-PR3 ANCAs is relatively rare. Our study suggests that GPA with nonidentified ANCA differs from GPA with identified ANCA by the frequency of localized forms., SCOPUS: ar.j, info:eu-repo/semantics/published

The aim of this study was to describe the clinical characteristics of ANCA-associated vasculitides (AAV) at presentation, in a wide cohort of Spanish patients, and to analyze the impact of the vasculitis type, ANCA specificity, prognostic factors, and treatments administered at diagnosis, in the outcome.A total of 450 patients diagnosed between January 1990 and January 2014 in 20 Hospitals from Spain were included. Altogether, 40.9% had granulomatosis with polyangiitis (GPA), 37.1% microscopic polyangiitis (MPA), and 22% eosinophilic granulomatosis with polyangiitis (EGPA). The mean age at diagnosis was 55.6 +/- 17.3 years, patients with MPA being significantly older (P < 0.001). Fever, arthralgia, weight loss, respiratory, and ear-nose-throat (ENT) symptoms, were the most common at disease onset. ANCAs tested positive in 86.4% of cases: 36.2% C-ANCA-PR3 and 50.2% P-ANCA-MPO. P-ANCA-MPO was significantly associated with an increased risk for renal disease (OR 2.6, P < 0.001) and alveolar hemorrhage (OR 2, P = 0.010), while C-ANCA-PR3 was significantly associated with an increased risk for ENT (OR 3.4, P < 0.001) and ocular involvement (OR 2.3, P = 0.002). All patients received corticosteroids (CS) and 74.9% cyclophosphamide (CYC). The median follow-up was 82 months (IQR 100.4). Over this period 39.9% of patients suffered bacterial infections and 14.6% opportunistic infections, both being most prevalent in patients with high-cumulated doses of CYC and CS (P < 0.001). Relapses were recorded in 36.4% of cases with a mean rate of 2.5 +/- 2.3, and were more frequent in patients with C-ANCA-PR3 (P = 0.012). The initial disease severity was significantly associated with mortality but not with the occurrence of relapses. One hundred twenty-nine (28.7%) patients (74 MPA, 41 GPA

Objective: To present a case of unusual presentations of granulomatosis with polyangitis (Wegener’s granulomatosis) with parotid swelling and intractable otitis externa. Material and method: A 22-year-old male patient presented with left sided otitis externa with left sided parotid swelling and bilateral nasal obstruction for last two weeks. CT scan of paranasal sinuses showed homogenous mass in both maxillary antra and nasal cavities. FNAC from the parotid swelling was suggestive of granulomatous disease and endoscopic biopsy from the nasal mass showed features of granulomatosis with polyangitis (Wegener’s granulomatosis). CT scan of chest revealed multiple cysts within the lung parenchyma; urine examination showed RBC and pus cells. Renal biopsy showed focal segmental glomerulonephritis. c-ANCA was highly positive and thus the diagnosis of Wegener’s granulomatosis was made. Result: Patient received cyclophosphamide and prednisolone immunsuppressive therapy for one year, which showed marked clinical improvement. Conclusion: Granulomatosis with polyangitis (Wegener’s granulomatosis) is not an uncommon entity for otolarygologists. Its usual presentation mimics chronic rhinosinusitis; but presentations like otitis externa and parotid swelling are rare for this disease.

Objective: To present a case of unusual presentations of granulomatosis with polyangitis (Wegener’s granulomatosis) with parotid swelling and intractable otitis externa. Material and method: A 22-year-old male patient presented with left sided otitis externa with left sided parotid swelling and bilateral nasal obstruction for last two weeks. CT scan of paranasal sinuses showed homogenous mass in both maxillary antra and nasal cavities. FNAC from the parotid swelling was suggestive of granulomatous disease and endoscopic biopsy from the nasal mass showed features of granulomatosis with polyangitis (Wegener’s granulomatosis). CT scan of chest revealed multiple cysts within the lung parenchyma; urine examination showed RBC and pus cells. Renal biopsy showed focal segmental glomerulonephritis. c-ANCA was highly positive and thus the diagnosis of Wegener’s granulomatosis was made. Result: Patient received cyclophosphamide and prednisolone immunsuppressive therapy for one year, which showed marked clinical improvement. Conclusion: Granulomatosis with polyangitis (Wegener’s granulomatosis) is not an uncommon entity for otolarygologists. Its usual presentation mimics chronic rhinosinusitis; but presentations like otitis externa and parotid swelling are rare for this disease.

Objective: To present a case of unusual presentations of granulomatosis with polyangitis (Wegener’s granulomatosis) with parotid swelling and intractable otitis externa. Material and method: A 22-year-old male patient presented with left sided otitis externa with left sided parotid swelling and bilateral nasal obstruction for last two weeks. CT scan of paranasal sinuses showed homogenous mass in both maxillary antra and nasal cavities. FNAC from the parotid swelling was suggestive of granulomatous disease and endoscopic biopsy from the nasal mass showed features of granulomatosis with polyangitis (Wegener’s granulomatosis). CT scan of chest revealed multiple cysts within the lung parenchyma; urine examination showed RBC and pus cells. Renal biopsy showed focal segmental glomerulonephritis. c-ANCA was highly positive and thus the diagnosis of Wegener’s granulomatosis was made. Result: Patient received cyclophosphamide and prednisolone immunsuppressive therapy for one year, which showed marked clinical improvement. Conclusion: Granulomatosis with polyangitis (Wegener’s granulomatosis) is not an uncommon entity for otolarygologists. Its usual presentation mimics chronic rhinosinusitis; but presentations like otitis externa and parotid swelling are rare for this disease.

Introduction: This study aims to elucidate the prognosis and the effectiveness of current treatments for Japanese patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). Methods: Patients with newly diagnosed MPA and GPA were enrolled in a nationwide, prospective, inception cohort study from 22 tertiary Japanese institutions, and treatment patterns and responses were evaluated for 24 months. Primary outcome measures were rates of remission (Birmingham Vasculitis Activity Score, 0) and remission with low-dose glucocorticoids (GC) (prednisolone ≤ 10 mg) (GC remission). Results: Of 156 enrolled patients, 78 MPA patients and 33 GPA patients were included. Concomitant cyclophosphamide (CY) was used in 24 MPA (31 %) and 20 GPA (60 %) patients during the initial 3 weeks of treatment. After 6 months, remission was achieved in 66 MPA (85 %) and 29 GPA (87 %) patients, while GC remission was obtained in only 31 MPA (40 %) and 13 GPA (39 %) patients. During the 24-month period, 14 MPA patients and 2 GPA patients died; end stage renal disease (ESRD) was noted in 13 MPA patients but no GPA patients. Patients with severe disease, according to the European Vasculitis Study Group (EUVAS) classification, showed poorer ESRD-free and overall survival rates than those with generalized disease (p < 0.0001). There were no differences in relapse-free survival rates between GPA and MPA, among EUVAS-defined disease severity categories, and between anti-neutrophil cytoplasmic antibody subspecialties. Conclusions: The majority of Japanese patients with MPA and GPA received treatment with high-dose GC and limited CY use, and showed high remission and relapse-free survival rates but low GC remission rates in clinical practice. Trial registration: University Hospital Medical Information Network Clinical Trials Registry UMIN000001648. Registered 28 February 2009.

Biopsies and ANCA testing for limited forms of granulomatosis with polyangiitis (GPA) are frequently non-diagnostic. We characterized gene expression in GPA and other causes of orbital inflammation. We tested the hypothesis that a sub-set of patients with non-specific orbital inflammation (NSOI, also known as pseudotumor) mimics a limited form of GPA. Formalin-fixed, paraffin-embedded orbital biopsies were obtained from controls (n=20) and patients with GPA (n=6), NSOI (n=25), sarcoidosis (n=7), or thyroid eye disease (TED) (n=20) and were divided into discovery and validation sets. Transcripts in the tissues were quantified using Affymetrix U133 Plus 2.0 microarrays. Distinct gene expression profiles for controls and subjects with GPA, TED, or sarcoidosis were evident by principal coordinate analyses. Compared with healthy controls, 285 probe sets had elevated signals in subjects with GPA and 1472 were decreased (>1.5-fold difference, false discovery rate adjusted p<0.05). The immunoglobulin family of genes had the most dramatic increase in expression. Although gene expression in GPA could be readily distinguished from gene expression in TED, sarcoidosis, or controls, a comparison of gene expression in GPA versus NSOI found no statistically significant differences. Thus, forms of orbital inflammation can be distinguished based on gene expression. NSOI/pseudotumor is heterogeneous but often may be an unrecognized, localized form of GPA.

OBJECTIVE: The long-term cancer risk for patients treated for granulomatosis with polyangiitis (GPA) is not well characterized. We assessed the risk of early and late-occurring cancers among 293 patients diagnosed with GPA from 1973 to 1999 and followed throughout 2010.METHODS: Cancer incidence in the cohort was determined by linkage with the Danish Cancer Registry and compared with that in the general population by calculation of standardized incidence ratios (SIRs).RESULTS: The median duration of follow-up was 9.7 years (range 0-36). Seventy-three cancers occurred, of which 30 were non-melanoma skin cancers (NMSCs) and 11 were bladder carcinomas. A high occurrence of NMSC was observed from the second year of follow-up onwards, with a SIR of 7.0 (95% CI 2.3, 16) for cases diagnosed ≥20 years after GPA. The incidence of bladder cancer increased after 5-9, 10-14 and 15-19 years of follow-up, with SIR estimates for these latency periods of 5.3 (95% CI 1.1, 15), 14.4 (95% CI 5.3, 31) and 10.5 (95% CI 1.2, 38), respectively. The incidence of myeloid leukaemia was significantly increased during years 5-9 [SIR 23.9 (95% CI 2.7, 86)]. Increased incidence of NMSC, bladder cancer and myeloid leukaemia was observed among patients exposed to cumulative CYC doses >36 g, while the only malignancy type observed in excess among those treated with lower CYC doses was NMSC. The cancer risk among CYC-naive patients was not significantly increased.CONCLUSION: GPA patients experience a greater than expected number of specific malignancies following conventional therapies. Our analyses demonstrate a substantially increased risk of very late-occurring NMSC and bladder cancer in this patient group.