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Microscopic polyangiitis (MPA) affects small and medium vessel, which sometimes leads to arterial aneurysms. In English database, only 15 reports refer to ruptured aneurysms in MPA. We experienced a fatal case with MPA who developed multiple visceral aneurysms, resulting in rupture of the hepatic aneurysm. For the better knowledge of aneurysmal rupture in MPA, we reviewed the feature of 16 cases, including our case. Organ involvement observed was glomerulonephritis 100%, pulmonary involvement 25%, peripheral neuropathy 25%, and purpura 12.5%. Locations of ruptured aneurysms were left gastric artery 31.25%, renal and hepatic artery 18.75% each, intracranial and splenic artery 12.5% each, and gastroepiploic and mesenteric artery 6.25% each. Median time to rupture was 45 days after systemic symptom onset, and 15 days after immunosuppressive treatment induction. Symptoms at rupture were visceral pain 68.75% and hemodynamic instability 62.5%. Pathological findings of ruptured aneurysms were acute vasculitis in 5, no evidence of active inflammation in 3. Causes of death were aneurysmal rupture in 5, treatment complications in 3, and total mortality rate was 50%. In conclusion, the initial presentation of MPA resulting in ruptured aneurysms tends to be renal-limited vasculitis. Aneurysms of abdominal medium-sized arteries tend to rupture, from 4 weeks after systemic symptom onset to 2 weeks after immunosuppressive treatment induction. Most aneurysms are less than 10 mm in diameter, develop asymptomatically in a few days, and are recognized when they rupture. Early induction of immunosuppressive treatment has the potential to shrink aneurysms and prevent rupture. [ABSTRACT FROM AUTHOR]

We compared different antineutrophil cytoplasmic antibody (ANCA) detection methods using a predominantly myeloperoxidase (MPO)-ANCA-associated vasculitis cohort. Stored sera from 147 patients with untreated ANCA-associated vasculitis (AAV), including microscopic polyangiitis and granulomatosis with polyangiitis (n = 115 and 32, respectively), and 124 disease controls were tested for P-ANCA and C-ANCA with immunofluorescence (IIF), and for MPO-ANCA and proteinase 3 (PR3)-ANCA with different antigen-specific immunoassays: direct enzyme-linked immunosorbent assay (ELISA), chemiluminescent enzyme immunoassay (CLEIA), third-generation fluorescent enzyme immunoassay (FEIA), and latex turbidimetrical immunoassay (LTIA). In addition, MPO-ANCA and PR3-ANCA titers were calibrated using certified reference materials (CRMs). The sensitivities and specificities for AAV diagnoses were 95% and 94% (IIF), 86% and 98% (ELISA), 93% and 94% (CLEIA), 92% and 96% (FEIA), and 68% and 88% (LTIA). Dual IIF/antigen-specific immunoassay testing reduced diagnostic accuracies from 94% to 93%. The quantitative agreement between ANCA levels measured using CLEIA and FEIA and calibrated using CRMs was not good. In conclusion, this study demonstrated the high performance of antigen-specific immunoassays for AAV diagnosis in a predominantly MPO-ANCA-associated vasculitis cohort and suggested that the benefit of dual IIF/antigen-specific immunoassay testing is limited. Standardizing ANCA measurements using different immunoassays was difficult, even when using CRMs. [ABSTRACT FROM AUTHOR]

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic necrotizing vasculitis that predominantly affects small vessels. In this report, we present a typical case of granulomatosis with polyangiitis (GPA) complicated by spontaneous renal hemorrhage (SRH), a rare but potentially severe condition. The patient developed SRH during immunosuppressive therapy but recovered following conservative treatment. We then conducted a systematic literature review on SRH in the context of AAV, and analyzed clinical features, management strategies, and patient prognosis. A total of 15 patients were enrolled for statistical analysis, comprising the one case reported in the current study and 14 from the literature. Of these patients, nine presented with GPA and six showed microscopic polyangiitis (MPA), with a sex distribution of 3:2 males to females. The average patient age was 54.5 years, and ranged from 25 to 82 years. Acute flank pain was the most common clinical manifestation, and was occasionally accompanied by anemia and shock. Treatment varied for the different patients. Eight patients received glucocorticoid and immunosuppressive agents that included rituximab, cyclophosphamide, and azathioprine; five patients underwent transcatheter arterial embolization (TAE); and one patient underwent nephrectomy. Our findings indicate that SRH typically occurs early in the course of AAV and correlates with disease activity, with renal aneurysm rupture as the primary cause. More than half of the patients respond well to corticosteroids and immunosuppressants. Timely TAE is essential for patients showing persistent deterioration despite conservative management. [ABSTRACT FROM AUTHOR]

Introduction: This study aimed to identify new clinical phenotypes of microscopic polyangiitis (MPA) using a principal components analysis (PCA)-based cluster analysis. Methods: A total of 189 patients with MPA between May 2005 and December 2021 were enrolled from a multicenter cohort in Japan (REVEAL cohort). Categorical PCA and cluster analysis were performed based on clinical, laboratory, and radiological findings. Clinical characteristics and outcomes, including all-cause mortality, respiratory-related mortality, end-stage renal disease (ESRD), and relapse were compared between each cluster. Results: Eleven clinical variables were transformed into four components using categorical PCA and synthetic variables were created. Additionally, a cluster analysis was performed using these variables to classify patients with MPA into subgroups. Four distinct clinical subgroups were identified: Cluster 1 included the renal involvements and diffuse alveolar hemorrhage (DAH)-dominant group (N=33). Cluster 2 comprised the elderly onset systemic inflammation group (N=75). Cluster 3 included patients in the younger-onset limited-organ disease group (N=45). Cluster 4 was comprised of an ILD-predominant group without kidney involvement (N=36). 61 patients died during follow-up, with 32 dying of respiratory-related causes. Additionally, 19 patients developed ESRD and 70 relapsed. Cluster 1 showed the worst ESRD-free survival and relapse rates, whereas Cluster 2 showed the worst overall survival and respiratory-related death-free survival rates among the four groups. Conclusions: Our study identified four unique subgroups with different MPA outcomes. Individualized treatments for each subgroup may be required to improve the prognosis of MPA. [ABSTRACT FROM AUTHOR]

Vasculitic neuropathies are a diverse group of inflammatory polyneuropathies that result from systemic vasculitis (e.g., polyarteritis nodosa, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis), vasculitis resulting from rheumatological disorders (e.g., rheumatoid arthritis and Sjögren's syndrome), paraneoplastic conditions, viruses, and medications. Occasionally, vasculitis is restricted to the peripheral nerves and termed nonsystemic vasculitic neuropathy. Presenting with an acute or subacute onset of painful sensory and motor deficits, ischemia to individual peripheral nerves results in the classic "mononeuritis multiplex" pattern. Over time, overlapping mononeuropathies will result in a symmetrical or asymmetrical sensorimotor axonal polyneuropathy. The diagnosis of vasculitic neuropathies relies on extensive laboratory testing, electrodiagnostic testing, and nerve and/or other tissue biopsy. Treatment consists primarily of immunosuppressant medications such as corticosteroids, cyclophosphamide, rituximab, methotrexate, or azathioprine, in addition to neuropathic pain treatments. Frequently, other specialists such as rheumatologists, pulmonologists, and nephrologists will comanage these complex patients with systemic vasculitis. Prompt recognition of these conditions is imperative, as delays in treatment may result in permanent deficits and even death. [ABSTRACT FROM AUTHOR]

Introduction: Multiple sclerosis (MS) is a chronic, disabling neurodegenerative disease, leads to reduced quality of life. The increasing prevalence of MS around the world and its comorbidities increase its burden. Primary vasculitis subtypes, one of autoimmune diseases with different prevalence in different ages and genders, should be considered one of the important differential diagnosis in patients with MS. This study aims to verify the relationship between MS and primary vasculitis by conducting a systematic review. Method: We searched PubMed, Scopus, EMBASE, Web of Science, and Google Scholar, from January 1974 to July 2023. We included original articles that reported characteristics of patients involved with any type of Primary Vasculitis with MS. Result: From an initial 816 publications, 18 studies consisting of 18 individual patients from 14 countries with confirmed MS and one of different subtypes of primary vasculitis met the inclusion criteria. The female/male ratio was 0.38:1, the mean (SD) age was 40.44 (14.37) years with the range of 16 to 70 years old, and the relapsing/progressive ratio was 1.57:1. Most of them, 14 (77%) experienced MS before vasculitis, and mostly received Corticosteroids, interferon, cyclophosphamide, Glatiramer acetate as MS treatment. The concurrence of Takayasu Arteritis (2 cases), Polyarteritis Nodosa (2 cases), Churg-Strauss Syndrome (1 case), Wegener's Granulomatosis (2 cases), Microscopic Polyangiitis (1 case), Cutaneous leukocytoclastic vasculitis (5 cases), Good pasture's disease (5 cases) were reported with MS. Conclusion: Our study suggested that different primary vasculitis can be an important comorbidity of MS and can mimic its symptoms and MRI. Any atypical syndrome for PwMS, whether clinical or radiological, must be evaluated in terms of other differential diagnoses including vasculitis. [ABSTRACT FROM AUTHOR]

Background/Objectives: Anti-neutrophil cytoplasmic antibodies (ANCAs) have been found in interstitial lung disease (ILD) in recent years, although its impact on ILD prognosis is less known. To date, ANCAs are not included in the interstitial pneumonia with autoimmune features (IPAF) definition criteria. Therefore, ANCA-ILD, in the absence of known ANCA-associated vasculitis (AAV), could be underdiagnosed. Our aim was to analyze the clinical profile and prognosis of ANCA-ILD patients. Methods: Patients diagnosed with ILD and positive ANCA were enrolled in a retrospective, monocentric cohort study. Lung function outcomes and mortality were assessed according to clinical, serological, radiological, and treatment characteristics. Survival was analyzed using Kaplan–Meier curves and Cox regression models. Results: A total of 23 patients were included, mostly women, with a median time from ILD diagnosis of 36 (24–68) months and a predominant anti-MPO pattern (56.5%). Nearly half of the patients had AAV, mostly microscopic polyangiitis (MPA). The presence of AAV was significantly associated with anti-MPO antibodies and an NSIP radiographic pattern. Overall, the fibrotic pattern (either UIP or fibrotic NSIP) was the most common (73.9%), mainly UIP (51.2%). However, it appeared less frequently in the AAV-ILD group. During follow-up, lung function impairment or radiological progression was observed in 65.2% of patients. Cumulative mortality incidence was high (43.4%), largely due to ILD itself (80%). A UIP pattern was associated with a higher and earlier mortality (HR 34.4 [1.36–132]), while the use of immunosuppressants showed a trend towards lower ILD-related death. Conclusions: In our cohort, ANCA-ILD patients mostly presented with fibrotic patterns, with AAV in almost half of the cases and a high and early mortality rate, which suggests the need to assess ANCA in all ILD patients. [ABSTRACT FROM AUTHOR]

Recent progress has notably improved outcomes for patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), namely granulomatosis with polyangiitis and microscopic polyangiitis. Since 2021, several international scientific societies have recommended rituximab (RTX) as the preferred primary treatment for maintaining remission in AAV patients. Decisions regarding retreatment with RTX are based on individual patient risk factors for disease flare-ups and the potential consequences of such flares. In reviewing available evidence and reporting our experiences at G. Bosco Hub Hospital in Turin, Italy, we explore various trials focusing on the maintenance therapy in AAV and discuss areas of unmet need. [ABSTRACT FROM AUTHOR]

Abstract Background This study investigated poor prognostic factors for the relapse of interstitial lung disease (ILD) in patients with microscopic polyangiitis (MPA) after remission induction therapy. Methods We enrolled patients diagnosed with MPA complicated by ILD according to the Chapel Hill Consensus definition from 2001 to 2023 in multiple institutions in the REVEAL cohort. All patients who were treated with immunosuppressive therapy were followed up, and those who relapsed with ILD were extracted in this study. We explored the risk factors for predicting ILD relapse in patients with MPA-ILD by comparing the demographic, clinical, laboratory, and radiological findings and treatments between the relapsed and non-relapsed groups on admission. Results Of 243 patients with MPA, 134 (55.1%) with MPA-ILD were enrolled. Among them, 28 (20.9%) relapsed during a mean follow-up of 4.2 years. The initial serum Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) levels and the prevalence of usual interstitial pneumonia (UIP) pattern were significantly higher in the relapsed group. The biomarkers were also risk factors for relapse in multivariate Cox regression analysis. The best cut-off values of KL-6, SP-D for predicting ILD relapse were 430 U/mL and 89.5 ng/mL, respectively. We created prediction models based on the best cut-off values for KL-6, SP-D, and the presence of the UIP pattern (KSU model). The 10-year relapse rate was significantly different among patients with MPA-ILD stratified by the number of risk factors based on the KSU model. A higher relapse rate was associated with higher all-cause mortality. Conclusions The initial serum high KL-6 and SP-D levels and the prevalence of the UIP pattern were associated with ILD relapse in patients with MPA-ILD. Our multicentre cohort study indicated that the KSU model, which consists of KL-6 ≥ 430 U/mL, SP-D ≥ 89.5 ng/mL, and the presence of the UIP pattern, is a useful predictor of ILD relapse in patients with MPA after immunosuppressive therapy.

Abstract Background The association between interstitial lung disease (ILD) and microscopic polyangiitis (MPA) has received increasing attention in recent years. However, there are still no studies comparing clinical characteristics and prognoses between MPA-ILD patients and patients with ANCA-negative ILDs. Therefore, the purpose of this study was to analyse a group of patients presenting MPA-ILD matched with patients exhibiting ANCA-negative ILDs to identify differences in their clinical characteristics and survival. Methods This study retrospectively reviewed the data of 60 patients with MPA-ILD and 60 patients with ANCA-negative ILDs who were matched for age, sex, and patterns on chest high-resolution CT (HRCT) images. The baseline clinical information, laboratory parameters and chest CT data were collected and analysed at each patient’s initial diagnosis. Results Sixty of the 682 (8.8%) ILD patients were diagnosed with MPA-ILD. MPA-ILD patients tended to be older and have higher CRP and ESR levels. ILD antedated MPA in 61.7% (37/60) of the patients, and MPA occurred on average (45.1 ± 33.4) months after the ILD diagnosis. Compared with matched ANCA-negative ILD patients, MPA-ILD patients had higher CRP and serum creatinine levels and lower haemoglobin levels. UIP (63.3%) was the most common chest HRCT pattern, and the proportion of patients with oddly shaped cysts (P = 0.04) was significantly greater in the MPA-ILD group than in the matched ANCA-negative ILD group. The number of MPA-ILD patients was significantly shorter than that of ANCA-negative ILD patients (P = 0.005). Survival analysis revealed that acute exacerbation (AE) of ILD (HR 2.40, 95% CI 1.03–5.59, P = 0.043) and diffuse alveolar haemorrhage (HR 3.42, 95% CI 1.09–10.73, P = 0.036) were independently associated with shorter survival and that receiving glucocorticoids combined with immunosuppressants (HR 0.11, 95% CI 0.03–0.37, P

Objective: To compare the performance of the EMA (European Medicines Agency) algorithm for classification of necrotizing vasculitis and the new American College of Rheumatology (ACR)/European League of Rheumatology (EULAR) 2022 classification criteria in our single center long-term anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) cohort. Methods: Patients classified as granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) according to EMA algorithm were included into the study. ACR/EULAR 2022 classification criteria were implemented retrospectively. Antibody-based classification (ABC) was performed as a third model, which classify patients either GPA or MPA if anti-proteinase 3 (PR3) or myeloperoxidase (MPO) is positive, respectively. Kappa analysis was used to explore the agreement between criteria sets. Results: Data of 221 patients classified as GPA (85.6%) and MPA (14.5%) according to EMA algorithm were included. PR3-ANCA and MPO-ANCA was positive in 124 (56.1%) and 79 (35.7%) patients. ACR/EULAR 2022 classified 137 (62%) and 84 (38%) patients as GPA and MPA, respectively. Nine (4%) patients were classified as both GPA and MPA, nine (4%) patients were unclassifiable. The new criteria set was in weak agreement with EMA algorithm (kappa=0.28 for GPA and 0.24 for MPA). On the other hand, strong agreement with ABC was observed (kappa=0.88 for GPA and 0.89 for MPA). Conclusion: A significant number of patients who classified as GPA could be classified as MPA with the ACR/EULAR 2022 criteria and agreement with EMA algorithm was weak. The new criteria set was indecisive for some AAV patients. Strong agreement with ABC indicated the significant influence of serology in the ACR/EULAR 2022 criteria.

Background: Pauci-immune glomerulonephritis (PIGN) is typically secondary to antineutrophil cytoplasmic antibodies (ANCA) small-vessel vasculitis. However, some cases lack detectable circulating ANCA and are called ANCA-negative PIGN (seronegative PIGN). The reported incidence of this varies greatly. Its relationship to ANCA-associated vasculitis (AAV) is unclear. Summary: This review explores the pathophysiology of seronegative PIGN and summarizes findings from 12 studies focusing on this disease. The role of neutrophils appears to be central, with activation through cellular and humoral mechanisms. Most studies have noted less extrarenal involvement and more chronic changes in the kidney biopsy in seronegative PIGN compared to ANCA-positive cases. Studies have mostly reported using corticosteroids with cyclophosphamide for induction therapy and azathioprine for maintenance. The renal survival was noted to be lower compared to ANCA-positive PIGN. Key Messages: Whether ANCA-negative PIGN represents a distinct disease or is part of the AAV spectrum remains unclear. Prospective large-scale studies are needed to understand this disease for optimal diagnosis and management.

Background: The association between interstitial lung disease (ILD) and microscopic polyangiitis (MPA) has received increasing attention in recent years. However, there are still no studies comparing clinical characteristics and prognoses between MPA-ILD patients and patients with ANCA-negative ILDs. Therefore, the purpose of this study was to analyse a group of patients presenting MPA-ILD matched with patients exhibiting ANCA-negative ILDs to identify differences in their clinical characteristics and survival. Methods: This study retrospectively reviewed the data of 60 patients with MPA-ILD and 60 patients with ANCA-negative ILDs who were matched for age, sex, and patterns on chest high-resolution CT (HRCT) images. The baseline clinical information, laboratory parameters and chest CT data were collected and analysed at each patient's initial diagnosis. Results: Sixty of the 682 (8.8%) ILD patients were diagnosed with MPA-ILD. MPA-ILD patients tended to be older and have higher CRP and ESR levels. ILD antedated MPA in 61.7% (37/60) of the patients, and MPA occurred on average (45.1 ± 33.4) months after the ILD diagnosis. Compared with matched ANCA-negative ILD patients, MPA-ILD patients had higher CRP and serum creatinine levels and lower haemoglobin levels. UIP (63.3%) was the most common chest HRCT pattern, and the proportion of patients with oddly shaped cysts (P = 0.04) was significantly greater in the MPA-ILD group than in the matched ANCA-negative ILD group. The number of MPA-ILD patients was significantly shorter than that of ANCA-negative ILD patients (P = 0.005). Survival analysis revealed that acute exacerbation (AE) of ILD (HR 2.40, 95% CI 1.03–5.59, P = 0.043) and diffuse alveolar haemorrhage (HR 3.42, 95% CI 1.09–10.73, P = 0.036) were independently associated with shorter survival and that receiving glucocorticoids combined with immunosuppressants (HR 0.11, 95% CI 0.03–0.37, P < 0.001) was independently associated with prolonged survival in MPA-ILD patients. Conclusions: The proportion of MPA-ILD patients with total ILD is not low, and ANCA testing of ILD patients is necessary. Oddly shaped cysts with a UIP pattern may be a characteristic chest imaging manifestation of MPA-ILD. The prognosis of MPA-ILD is poor, especially for patients who are older, have DAH, and have experienced AEs. [ABSTRACT FROM AUTHOR]

Background: Heredity and epigenetics affect the pathogenesis of microscopic polyangiitis (MPA). Tyrosine kinase 2 (TYK2) polymorphisms (rs2304256C > A, rs280519A > G, and rs12720270G > A) may be potential protective factors against anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Current research suggests that TYK2 is associated with various autoimmune diseases; however, no study has examined the relationship between TYK2 polymorphisms and AAV. This study assessed the effect of TYK2 polymorphisms on susceptibility to MPA. Methods: Overall, 562 Chinese participants (265 patients with MPA and 297 healthy volunteers) were recruited. Polymerase chain reactions combined with high-throughput sequencing were used to analyze polymorphic loci, while logistic regression analysis was used to assess the relationship between polymorphism of the TYK2 gene and MPA susceptibility. Results: In males, individuals with the CA genotype (rs2304256) in the overdominant model showed a significantly reduced risk of MPA (odds ratio (OR) = 0.52; 95% confidence interval (CI) [0.29–0.93]; p = 0.025). Regarding rs280519, male carriers of the AG genotype had a significantly lower risk of developing MPA in both the codominant (OR = 0.51; 95% CI [0.28–0.93]; p = 0.039) and overdominant (OR = 0.48; 95% CI [0.27–0.86]; p = 0.013) models. The GA genotype of rs12720270 was associated with low susceptibility to MPA in males (OR = 0.52; 95% CI [0.29–0.93]; p = 0.027). Conclusions: This study indicates that mutations in the TYK2 gene (rs2304256, rs280519, and rs12720270) may be associated with a reduced risk of MPA in the male Chinese population in Guangxi. The A allele of single nucleotide polymorphism (SNP) rs2304256 may be a protective factor against MPA, while the G alleles of SNPs rs280519 and rs12720270 are protective factors against MPA. [ABSTRACT FROM AUTHOR]

Although rapidly progressive glomerulonephritis (RPGN) is the main renal phenotype of microscopic polyangiitis (MPA), we aim to clarify the clinical features of slowly progressive MPA. This retrospective observational study included 12 patients diagnosed with MPA in our hospital between January 2012 and February 2022. We investigated the differences in surrogate markers, rate of decline of estimated glomerular filtration rate (eGFR) between the slowly progressive and rapidly progressive MPA groups. Of the 12 patients with MPA, 3 (25.0%) had slowly progressive MPA: MPA within 30% decrease in eGFR 3 months pretreatment, all of whom developed RPGN during the course. Patients with slowly progressive MPA had lower levels of C-reactive protein, myeloperoxidase anti-neutrophil cytoplasmic antibodies, and interleukin-6; higher levels of sialylated carbohydrate antigen KL-6. Slowly progressive MPA is not uncommon in our hospital. A linear relationship was found between slower rate of eGFR decline and lower surrogate markers of disease activity. Some MPA cases have slowly progressive glomerulonephritis leading to RPGN, which may be clinically characterized by low disease activity. It may be useful to measure myeloperoxidase anti-neutrophil cytoplasmic antibody in chronic kidney disease with concomitant urinary abnormalities to diagnose MPA with slowly progressive glomerulonephritis. KEY LEARNING POINTS: Rapidly progressive glomerulonephritis is the main renal phenotype of microscopic polyangiitis (MPA), and slowly progressive MPA is rarely observed. Slowly progressive MPA was not rare in our hospital and was characterized clinically by low disease activity and complicated by interstitial pneumonia. When encountering patients with undiagnosed chronic kidney disease complicated by interstitial pneumonia, measuring myeloperoxidase anti-nuetrophil cytoplasmic antibody regardless of the rate of renal function decline, potentially leads to the diagnosis of slowly progressive MPA. [ABSTRACT FROM AUTHOR]

The document provides a comprehensive overview of the diagnosis, monitoring, and treatment of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) with renal involvement, focusing on granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). It outlines the definitions, clinical presentation, histopathological classification, monitoring strategies, induction and maintenance treatments, as well as special considerations for relapsing, refractory, and frail patients with renal AAV. The document was prepared by the Catalan Group for the Study of Glomerular Diseases (GLOMCAT), which comprises nephrologists with extensive experience in the diagnosis and treatment of AAV patients. Several virtual and face-to-face meetings were held for coordination, section assignments, and content discussion. An exhaustive and systematic search of the literature was carried out, which included, among others, the following databases: PubMed, EMBASE, Cochrane Library, Google Scholar, and ClinicalTrials.gov, as well as the abstract books of national and international congresses. Overall, the document provides a comprehensive guide for clinicians managing patients with renal AAV, offering evidence-based recommendations for diagnosis, monitoring, and treatment across various clinical scenarios. [ABSTRACT FROM AUTHOR]

Objectives: Pneumocystis jirovecii pneumonia (PJP) is a serious complication of autoimmune and inflammatory diseases. This study aimed to describe the characteristics of PJP in patients with various systemic vasculitides and explore potential prognostic factors. Method: Data on 62 enrolled PJP patients with systemic vasculitis were analyzed. Patients were stratified based on the outcomes. Prognostic factors were investigated using Cox-regression models. Characteristics of patients with and without interstitial lung disease (ILD) were compared. Results: Among 62 vasculitis-PJP patients, 48 had anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), with microscopic polyangiitis (MPA) being the most common subtype (28 patients). MPA (HR 4.33, p = 0.001), concomitant aspergillosis (HR 2.68, p = 0.019), and higher D-dimer at PJP diagnosis (HR 1.07, p = 0.004) were independent adverse prognostic factors for overall survival. Stable disease activity of vasculitis was an independent favorable prognostic factor (HR 0.28, p = 0.027). Patients with MPA were older than non-MPA patients (median age: 69 vs. 58 years, p = 0.001); both ILD and fibrotic ILD were more prevalent in MPA patients (ILD: 78.6% vs. 35.3%, p = 0.001; fibrotic ILD: 57.1% vs. 11.8%, p < 0.001). At the diagnosis of PJP, patients with preexisting ILD had higher counts of white cells, lymphocytes, and neutrophils, as well as higher levels of immunoglobulin (Ig) G and IgA, than patients without preexisting ILD. Conclusions: MPA was associated with a higher risk of death in patients with vasculitis-PJP, possibly due to a higher prevalence of ILD. In clinical practice, we should pay more attention to the prophylaxis and management of PJP in patients with systemic vasculitis-associated ILD and/or MPA. Key Points • Data from this study showed that MPA was the most common subtype of vasculitis among vasculitis-PJP patients. • Compared with non-MPA patients in this study, patients with MPA were older, had more ILD and fibrotic ILD, and had a poorer prognosis. • In clinical practice, we should pay more attention to the prophylaxis and management of PJP in patients with systemic vasculitis-associated ILD and/or MPA. [ABSTRACT FROM AUTHOR]

Microscopic polyangiitis (MPA) is a form of necrotizing vasculitis affecting the small vessels. Our study is the first study with the objective of describing the difference in clinical presentation of MPA and response to treatment at 6 months based on the age of disease onset. All patients seen at a tertiary vasculitis clinic between 2015 and 2023 with a diagnosis of MPA were included. Patients were divided into an elderly group (age > = 65 years) and a younger group (age < 65). Comparative analysis was conducted to characterize differences amongst the elderly and younger patients, including differences in organ involvement and clinical presentation, Birmingham Vasculitis Activity Score (BVAS) scores, Vasculitis Damage Index(VDI) scores, and response to treatment at 6 months. Thirty-one patients were included in our study. Younger MPA patients (n = 18) with mean age at diagnosis of 53.17 years were compared with older MPA patients(n = 13) with mean age at diagnosis of 76.08 years. The younger patients had statistically significant higher BVAS scores (p = 0.009), along with higher incidence of renal (p = 0.028), pulmonary (p = 0.0069), and cutaneous (p = 0.026) manifestations at disease onset. Furthermore, amongst the younger population, there was noted statistically significant clinical improvement at 6 months following treatment induction, particularly in the domains of general symptoms (p = 0.011), MSK (p = 0.019), cutaneous (p = 0.011), and pulmonary symptoms (p = 0.04). In contrast, the elderly population presented with a predominant of non-specific constitutional symptoms, with statistically significant improvement in the domain of non-specific general symptoms at 6 months (p = 0.00008). All MPA patients responded well to treatment, with statistically significant improvement amongst younger patients (p = 0.0032), but no statistically significant improvement amongst elderly patients (p = 0.67). Our study findings concluded that MPA's clinical presentation differed by age group. Younger patients had more aggressive vasculitis disease presentation with better response to treatments, whereas, elderly patients had less severe disease presentation with predominant of general symptoms at disease onset. Key Points • MPA clinical presentation differed by age at disease onset. Younger patients presented with more aggressive and classic vasculitis-like presentations, with multi-system organ involvement and statistically significant higher incidence of renal, pulmonary, and cutaneous manifestations. In contrast, elderly patients had a predominant of constitutional and non-specific symptoms with often delayed diagnosis. • All MPA patients responded well to treatment. Amongst the younger population, there was statistically significant improvement in BVAS scores after treatment induction at 6 months; however, there was no statistically significant improvement of BVAS scores in the elderly population. • Future studies are required to further understand the difference in the clinical presentation of MPA based on the age at disease onset, and the implication on disease diagnosis and management. [ABSTRACT FROM AUTHOR]

Objective: Infections are a critical concern for patients with microscopic polyangiitis (MPA). This study aimed to identify the risk factors associated with serious infections (SIs) and infection-related mortality in patients with MPA, as well as the effect of glucocorticoid (GC) dose tapering on these outcomes. Methods: This multicentre, retrospective, and observational study utilised data from a cohort of patients with MPA in Japan [Registry of Vasculitis Patients to Establish REAL World Evidence (REVEAL) cohort]. Patients were categorised based on the occurrence of SIs or infection-related deaths, and various characteristics were compared among the groups. Results: Among 182 patients, 66 (36.2%) experienced 129 SIs and 27 (14.8%) developed infection-related deaths. Advanced age, elevated C-reactive protein (CRP) levels, and higher ratio of the GC dose at 3 months to the initial dose were identified as independent risk factors for SIs. Older age was also associated with infection-related deaths. Furthermore, the cumulative incidence of infection-related deaths was significantly higher in patients with a higher ratio of the GC dose at 24 months to the initial dose. Conclusion: Older age, elevated CRP levels, and slower GC dose tapering predispose patients to SIs and infection-related deaths. Strategies, such as rapid GC dose tapering, are anticipated to mitigate the risk of infections. [ABSTRACT FROM AUTHOR]

ANCA-associated vasculitides (AAV) comprise granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis. All forms may involve different organ systems, yet kidney and lung involvement are common and fatal in many cases. Here, we aimed to determine the predictive value of pulmonary disease manifestation and individual CT findings in AAV patients. Available CT scans and clinical information on mortality, renal outcomes, occurrence of relapses and damage scores were analysed retrospectively from a tertiary rheumatology center in Germany. We included a total of 94 AAV patients (49 with GPA, 41 with MPA). Forty-four patients had lung involvement with available CT scans, 70.5% of which with GPA and 72.7% with renal involvement. Nodule formation and cavities were more frequent among GPA patients, whereas ground-glass opacities (GGO), ILD and pleural effusion were observed predominantly in MPA patients. Over a median follow-up of 37 months, GPA patients had a slightly higher overall mortality, whereas end-stage kidney failure rates were significantly increased in MPA patients. Relapse frequencies were comparable between both entities. The presence of GGO and pleural effusion were associated with higher relapse rates, whereas nodules were negatively correlated with relapses. Notably, RTX-treated patients had less infections as compared to individuals under different therapies. Our data demonstrate the outstanding importance of characteristic CT patterns in AAV diagnosis assessment. Especially certain CT patterns including GGO and pleura effusion may help to identify patients who are at higher risk for relapsing disease. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to investigate whether the serum extracellular newly identified receptor for advanced glycation end products binding protein (EN-RAGE) and the soluble form of RAGE (sRAGE) measured at diagnosis are associated with all-cause mortality in patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). Materials and Methods: Serum EN-RAGE and sRAGE were measured in 75 immunosuppressive drug-naïve MPA and GPA patients using an immunoassay, with their clinical and laboratory data reviewed. The optimal cut-off point of EN-RAGE and sRAGE was calculated by finding the threshold with the maximum sum of sensitivity and specificity. In addition, the least absolute shrink-age and selection operator regression was adopted to select variables included in the multivariable Cox proportional hazards (PH) regression model. Results: The median age of the patients was 67.0 years, and 34% were male. Neither serum EN-RAGE nor sRAGE at diagnosis was correlated with the Birmingham Vasculitis Activity Score. Furthermore, no correlation was observed between serum EN-RAGE and sRAGE. Deceased patients had significantly lower serum EN-RAGE and higher serum sRAGE at diagnosis compared to surviving patients. Patients with serum EN-RAGE at diagnosis ≤84.37 ng/mL and serum sRAGE at diagnosis ≥1.82 ng/mL showed significantly lower survival probabilities compared to those without. In multivariable Cox PH regression model, only serum sRAGE at diagnosis ≥1.82 ng/mL, rather than serum EN-RAGE at diagnosis ≤84.37 ng/mL, was independently associated with all-cause mortality (hazard ratio 7.094). Conclusion: This study is the first to demonstrate that serum sRAGE at diagnosis may independently predict all-cause mortality during follow-up in patients with MPA and GPA. [ABSTRACT FROM AUTHOR]

Objective To compare the performance of the EMA (European Medicines Agency) algorithm for classification of necrotizing vasculitis and the new American College of Rheumatology (ACR)/European League of Rheumatology (EULAR) 2022 classification criteria in our single center long-term anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) cohort. Methods Patients classified as granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) according to EMA algorithm were included into the study. ACR/EULAR 2022 classification criteria were implemented retrospectively. Antibody-based classification (ABC) was performed as a third model, which classify patients either GPA or MPA if anti-proteinase 3 (PR3) or myeloperoxidase (MPO) is positive, respectively. Kappa analysis was used to explore the agreement between criteria sets. Results Data of 221 patients classified as GPA (85.6%) and MPA (14.5%) according to EMA algorithm were included. PR3-ANCA and MPO-ANCA was positive in 124 (56.1%) and 79 (35.7%) patients. ACR/EULAR 2022 classified 137 (62%) and 84 (38%) patients as GPA and MPA, respectively. Nine (4%) patients were classified as both GPA and MPA, nine (4%) patients were unclassifiable. The new criteria set was in weak agreement with EMA algorithm (kappa=0.28 for GPA and 0.24 for MPA). On the other hand, strong agreement with ABC was observed (kappa=0.88 for GPA and 0.89 for MPA). Conclusion A significant number of patients who classified as GPA could be classified as MPA with the ACR/EULAR 2022 criteria and agreement with EMA algorithm was weak. The new criteria set was indecisive for some AAV patients. Strong agreement with ABC indicated the significant influence of serology in the ACR/EULAR 2022 criteria. Keywords:ANCA associated vasculitis, microscopic polyangiitis, granulomatosis with polyangiitis, classification criteria: [ABSTRACT FROM AUTHOR]

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Objective. This study aimed to compare the 2022 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria with the European Medicines Agency (EMA) algorithm for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Methods. All consecutive, newly diagnosed patients with AAV according to the 2012 Chapel Hill Consensus Conference who visited Keio University Hospital between March 2012 and May 2022 were retrospectively reviewed. Patients were reclassified according to the EMA algorithm and the 2022 ACR/EULAR criteria, and their clinical characteristics were statistically analyzed. Results. A total of 114 patients with AAV were included in the analyses. Using the EMA algorithm as a reference, reclassification of the patients revealed sensitivity and specificity of the 2022 ACR/EULAR criteria of 100% and 96% for eosinophilic granulomatosis with polyangiitis, 40% and 97% for granulomatosis with polyangiitis (GPA), and 90% and 49% for microscopic polyangiitis (MPA), respectively. Approximately half of patients classified as EMA-GPA or EMA-unclassifiable were reclassified as 2022-MPA; these patients were older, were more disposed to be positive for myeloperoxidase (MPO)-ANCA, and had interstitial lung disease (ILD) more frequently than patients with 2022-GPA or non--2022-MPA. Further, some patients positive for MPO-ANCA with biopsy-proven granulomatous inflammation were also reclassified from EMA-GPA to 2022-MPA. Over the mean observation period of 4.0 years, 16 patients died. Overall survival for each classification group differed significantly from the 2022 ACR/EULAR criteria (P = 0.02), but not with the EMA algorithm (P = 0.21). Conclusion. Among the patients classified as EMA-GPA or EMA-unclassifiable, older patients with MPO-ANCA and ILD tended to be reclassified as 2022-MPA. The 2022 ACR/EULAR criteria were more useful in prognostic prediction than the EMA algorithm. [ABSTRACT FROM AUTHOR]

Objectives: Ceruloplasmin is an inhibitor of myeloperoxidase (MPO) activity that plays an important role in the pathophysiology of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). This study aimed to evaluate the prognostic impact of serum level of ceruloplasmin at diagnosis in patients with anti-MPO antibody-positive AAV. Methods: This retrospective monocentric study in Caen University Hospital involved all consecutive adult anti-MPO antibody-positive patients with microscopic polyangiitis or granulomatosis with polyangiitis, diagnosed between January 2010 and January 2022 with available serum sample at inclusion. Patients outcomes were analyzed from two subgroups constituted according to the median serum level of ceruloplasmin. The same analyses were then performed in anti-proteinase 3 (PR3) antibody-positive patients. Results: Within the 92 patients analyzed, 50 patients had anti-MPO antibodies with a median ceruloplasmin level of 0.44 [quartiles 1–3, 0.40–0.49] g/L and a median Birmingham Vasculitis Activity Score of 19 [14–22]. After a median follow-up period of 40 [22–86] months, 13 (26%) patients had died: 10 (40%) in the low ceruloplasmin group and 3 (12%) in the high ceruloplasmin group (p = 0.03), with a significantly worse survival rate in the low ceruloplasmin group (p = 0.021). No significant differences in relapse rate or renal failure was observed between the two groups. The same analyses performed in the group of AAV patients with anti-PR3 antibody did not show any differences. Conclusion: In anti-MPO AAV patients, serum level of ceruloplasmin at diagnosis seems to be associated with a significant impact on survival. [ABSTRACT FROM AUTHOR]