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Copyright of Journal of the Argentine Society of Rheumatology/Revista de la Sociedad Argentina de Reumatología is the property of Editorial Biotecnologica S.R.L and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

We present the case of a 36-year-old woman with a history of granulomatosis with polyangiitis, chronic kidney disease, and systemic arterial hypertension. Debut with dyspnea, weakness, and hemoptysis, she was suspected in atypical pneumonia, discarded, persisting with tachypnea, tachycardia, and chest pain. The protocol for pulmonary tuberculosis was started with negative sputum samples, positive blood culture for Staphylococcus haemolyticus , chest tomography with left pneumothorax and ipsilateral pleural effusion, exudate-type pleural fluid was obtained, acid-fast staining, negative PCR for Mycobacterium tuberculosis. A follow-up echocardiogram was performed due to a new murmur, reporting valvular vegetation, concluding a diagnosis of pleural tuberculosis and endocarditis as complications of multifactorial origin associated with immunosuppression in granulomatosis with polyangiitis. [ABSTRACT FROM AUTHOR]

Copyright of Acta Medica Colombiana is the property of Acta Medica Colombiana and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introduction: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare clinical entities with broad manifestations that are associated with diagnostic challenges. Prompt treatment is associated with significant prognostic improvement. Case report: 45-year-old female presenting with constitutional symptoms of 2 months of evolution, associated in the last 2 weeks with progressive hypoacusis. Acute renal lesion associated with active urinary sediment data was evidenced. The requested immune profile was positive for ANCA PR-3, and the renal biopsy confirmed the diagnosis of Granulomatosis with Polyangiitis. Immunosuppressive treatment with cyclophosphamide was started, with little improvement in renal function and requiring chronic hemodialysis. The wide variety of clinical manifestations associated with ANCA vasculitis lead to the inability to diagnose them early and hypoacusis, although described, is a rare initial manifestation. Conclusion: AAV are rare clinical entities and when they present with atypical manifestations, they are often a diagnostic challenge. It is imperative to consider it in cases of constitutional symptoms, associated with renal, otorhinolaryngologic and pulmonary manifestations. [ABSTRACT FROM AUTHOR]

Granulomatosis with polyangiitis (GPA) is an autoimmune vasculitis which rarely affects the lower genitourinary tract. We share the case of a 53-year-old man who presented with a retroperitoneal mass and thereafter developed a left multiseptated hydrocele that conditioned a testicular infarction. The pathology report of the orchidectomy was consistent with GPA. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To determine the otological manifestations of patients with granulomatosis with polyangiitis. MATERIALS AND METHODS: A descriptive, cross-sectional, analytical and observational study of the otological, nose and throat manifestations in patients with granulomatosis with polyangiitis, done from July to November 2019 at National Medical Center La Raza, Mexico City. RESULTS: Twenty patients with the diagnosis of granulomatosis with polyangiitis, 12 women and 8 men, mean age of 53 years, were evaluated. Otological manifestations were: aural fullness (14/20), tinnitus (12/20) and hearing loss (11/20). CONCLUSIONS: Average age of granulomatosis with polyangiitis involvement was the fifth decade of life, predominantly in women, all patients presented otorhinolar-yngological manifestations, highlighting different degrees of hearing loss in half of the population. There are significant associations of inner and middle ear manifestations. The complete otorhinolaryngological examination is important for the control and limitation of sequelae produced by this disease. [ABSTRACT FROM AUTHOR]

Copyright of Cirugía y Cirujanos is the property of Publicidad Permanyer SLU and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background/aim: Abnormal capillaroscopic findings have been reported in vasculitic syndromes such as Behçet's disease, Henoch- Schönlein purpura, and Wegener's granulomatosis. This study was conducted to define the capillaroscopic changes in patients with Takayasu arteritis (TA). Materials and methods: We studied 28 TA patients (27 females). The nail folds from the 2nd to 5th fingers on both hands were examined with video capillaroscopy for all. A patient was defined as having an abnormal capillaroscopic examination if more than 1 morphologic abnormality was present in at least 2 nail folds. Results: The median capillary density of TA patients was 9 (range: 9-11). There were no patients with capillary disorganization or avascular areas. Tortuous capillaries were detected in all patients. The other common morphological capillary abnormalities included enlarged/dilated capillaries (39.3%), branching capillaries (35.7%), and hemorrhages (32.1%). Only 1 patient had giant capillaries with early scleroderma-like pattern. Overall, there were 11 (39.3%) patients with abnormal capillaroscopic findings. There were more patients with abnormal capillaroscopic findings in the subgroup of TA patients whose upper extremity blood pressure could not be measured as compared to those whose blood pressure could be measured (66.7% vs. 26.3% patients; P = 0.04). Conclusion: Capillaroscopic abnormalities are frequently seen in TA patients. We consider that abnormal capillaroscopic findings in TA patients reflect the impaired blood flow due to narrowed or occluded arteries rather than the primary capillary involvement of the disease process. [ABSTRACT FROM AUTHOR]

It presents a patient who comes to Otorhinolaryngology Consultation with abundant nasal and secretions and fetids, nasal obstruction, increased volume of the nose with tumor appearance and pain of one year of evolution; a previous biopsy only showed necrosis. Treatment was imposed but it did not improve. Presented a greater nasal obstruction and a new nasal mucosa biopsy was performed; it was diagnosed as a Wegener's granulomatosis. The interesting thing about the case lies in the difficulty in making the diagnosis because clinically it simulated a tumor and in the biopsies the necrosis predominated, with only a few areas of granulomas. [ABSTRACT FROM AUTHOR]

ANCA-associated vasculitides at Mexico City's metropolitan eastern area Background: The anti-neutrophilcytoplasmic antibody-associated (ANCA) vasculitides includes granulomatosis with polyangiitis (Wegener's) (GPW), Chrug-Strauss syndrome (CSS) and microscopic polyangiitis (MPA). Since it has low incidence in our field, there are a few published papers. The aim of this study was to report the clinical characteristics, activity and damage of these vasculitides in Mexico City's metropolitan eastern area. Methods: We studied 28 cases with ANCA-associated vasculitis in the aforementioned area. The classification was made according to the ACR 1990 criteria, the Chapel Hill 2012 consensus, and the EULAR 2007 recommendations. We collected demographic data, as well as data of clinical manifestations, laboratory, management and outcomes. The activity was established using the Birmingham Vasculitis Activity Score (BVAS) and the damage using the Vasculitis Damage Index (VDI). We used paired t test and 95% confidence intervals (95% CI). Results: 15 females and 13 males were included, 20 with GPW and 8 with CCS. The mean age was 50.1 ± 12.7 years, and the evolution time was 69 ± 49.6 months. We didn't find cases of MAP. 23 patients (82.1 %) had general symptoms. The initial and final BVAS means were 10.5 and 1.5 (p < 0.001) and the global VDI was 2.4. Conclusions: The presence of ANCA-associated vasculitides is rare in the eastern Metropolitan area of Mexico City. GPW was predominant over CCS, and MPA was absent. They are multi-system vasculitides with high scores of initial activity and moderate damage. [ABSTRACT FROM AUTHOR]

Background: Granulomatosis with polyangiitis (GP) is a systemic necrotizing vasculitis with multi-organ involvement that primarily affects the respiratory tract and the kidneys. Fever in these patients is an indicator of activity; however, if it arises in isolation, the physician should exclude other causes. Clinical case: Male patient admitted due to an unexplained fever and weight loss; it was diagnosed GP by a history of pauci-immune glomerulonephritis, fixed pulmonary nodules and chronic sinusitis of four years of evolution; however, the fever persisted despite treatment and in the absence of infection. It was performed an oropharynx lesion biopsy and the diagnosis was extranodal NK-T-cell lymphoma, nasal type, and positive for CD56 and granzyme. Conclusion: Extranodal NK-T-cell lymphoma, nasal type is a rare entity, of poor prognosis, that should be considered as a diagnosis in patients with GP unresponsive to steroid. That is the reason why biopsy of the lesion and immunohistochemistry are required. [ABSTRACT FROM AUTHOR]

Introduction: Subglottic stenosis (SGS) in granulomatosis with polyangiitis (GPA) may result from active disease or from chronic recurrent inflammation. The objective of the study was to describe the clinical features and treatment of patients with subglottic stenosis. Methods: We retrospectively reviewed the medical records of all patients with SGS due to GPA diagnosed at Rheumatology deparment between January 2000 and June 2015. Results: We present 4 cases of SGS at our department during a period of 15 years. The interval between the presentation of the GPA and SGS varied between 2 and 144 months. The leading symptoms of SGS were dyspnoea on exertion and stridor. Three patients presented SGS without evidence of systemic activity. Two patients presented SGS grade i and received tracheal dilatation; two recurred and three needed a tracheostomy due to severe airway-limiting stenosis. Conclusion: SGS presents high morbidity. Even though subglottic dilatation provides symptomatic relief, recurrences may present. Severe airway-limiting stenosis often requires tracheostomy. [ABSTRACT FROM AUTHOR]

ANCA-associated vasculitides (AAV) are chronic autoimmune diseases characterized by inflammation and destruction of small vessels. Rituximab is now licensed for use as a remission-induction agent in the treatment of these disorders. During recent years, several non-controlled studies have suggested that rituximab may be of value in maintaining disease remission in AAV. In these series, 3 techniques have been tried: "watch-and-wait", repeated cycles in fixed intervals, or administration based on proposed biomarkers. More importantly, the results of the MAINRITSAN trial showed that this anti-CD20 agent is superior to azathioprine for preventing major relapses in AAV. This review summarizes current information regarding the effectiveness, timing, dosing, duration and safety o1f rituximab as a valid option for remission maintenance. [ABSTRACT FROM AUTHOR]

Vasculitis is a group of diseases of unknown etiology, whose main characteristic is the gradual occlusion of the arterial lumen to a total occlusion of the same, which affects a variety of organs, that include lungs, kidneys, skin and brain, among others. Its pathophysiology is partially known; however, it is known that there is a disturbance of the immune system in which autoantibodies are produced, many of them are directed against multiple antigens located in the walls of blood vessels. Main autoantibodies produced are the ANCAs (anti-neutrophil cytoplasmic antibodies). Their role is mainly an indication in the diagnosis of this disease because it allows classifying positive (Wegener's granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis) and negative ANCA vasculitis (Henoch-Schönlein purpura, cryoglobulinemic vasculitis, Kawasaki disease, Takayasu's arteritis and temporal arteritis). [ABSTRACT FROM AUTHOR]

Objective: To describe the clinical and laboratory data, with special emphasis on thoracic imaging findings, in 14 patients with a definitive diagnosis of granulomatosis with polyangiitis (GPA). Methods: The clinical and tomographic data of 14 patients with a definitive diagnosis of GPA are presented. Patients with thoracic manifestations suggestive of GPA were evaluated in 3 hospitals from 2000 to 2012. All patients had a sputum analysis and bronchoalveolar lavage for bacterial, mycobacterial and fungal stains and cultures; antineutrophil cytoplasmic antibodies, antinuclear-antibodies, rheumatoid factor, and a biopsy of involved organs. Results: A total of 13 patients had at least two organs involved. The most frequent thoracic findings were pulmonary nodules, ground glass opacities and patches of consolidation; other abnormalities were tracheal stenosis, diffuse alveolar hemorrhage, lung masses with organized pneumonia. More than three- quarters (78%) of patients had positive antineutrophil cytoplasmic antibodies (ANCA). Ten patients had respiratory tissue biopsy (8 open lung, one tracheal, and one nasal). In 4 patients the diagnosis was made with the classic organ involvement in GPA, positive ANCA, and renal or skin biopsy, and response to treatment on follow-up. At 6-12 months all patients showed clinical and radiological improvement, with 54% showing a recurrence of disease. Discussion: The majority of thoracic findings described in GPA are presented in this study. A complete diagnostic approach with invasive diagnostic procedures to rule out other more prevalent respiratory diseases with similar thoracic manifestations must be performed. The positivity of ANCA in this study was high, and the recurrence of the disease was frequent. [ABSTRACT FROM AUTHOR]

Hypertrophic pachymeningitis is a disorder of the duramater that causes focal or diffuse thickening. It occurs predominantly in autoimmune, neoplastic and infectious diseases. When the cause is not possible to be established this condition is known as idiopathic hypertrophic pachymeningitis (IHP). Clinically there is involvement of cranial nerves, cerebellar symptoms and paraparesis. The imaging features include isodense (computed tomography scan) or hypointense (magnetic resonance imaging) appearance with great enhancement after the administration of contrast, as well as meningeal thickness exceeding 2 mm. Treatment is focused to correct the cause, and when considering IHP, steroids or low-dose methotrexate could be useful. [ABSTRACT FROM AUTHOR]

Male with diagnosis of Wegener's granulomatosis associated to anti-proteinase 3 antibodies that improved initially to the treatment with high dose glucocorticoids and ciclophosphamide, but in a relapse he did not have good response to glucocorticoid treatment, ciclophosphamide, methotrexate nor azathioprine. The patient received treatment with rituximab in 4 doses with clinical and radiographic improvement. [ABSTRACT FROM AUTHOR]