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Several studies have examined the utility of quadriceps femoris muscle biopsies for diagnosing antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We aimed to investigate the effects of glucocorticoid therapy on the muscle biopsy findings of patients with AAV. Data from patients with AAV who underwent a biopsy of the bilateral vastus lateralis of the quadriceps femoris at Tokyo Metropolitan Tama Medical Center between January 2015 and December 2023 were retrospectively analyzed. In total, 126 patients with suspected small vessel vasculitis (SVV) or medium vessel vasculitis (MVV) underwent a muscle biopsy at the study center. Of these, the present study included 71 patients with a final diagnosis of AAV who received a muscle biopsy of the bilateral vastus lateralis at disease onset and began glucocorticoid therapy at the study center. Of the 71 patients, 41 (57.7%) had a positive muscle biopsy finding as defined by the presence of inflammatory infiltrates with fibrinoid necrosis in the vessels. Fifty-six and 15 patients underwent a biopsy before and after starting glucocorticoid therapy, respectively. Of the former, 33 (58.9%) had a positive finding compared to eight (53.3%) of the latter (Pearson's chi-square test: p = 0.697). Among the patients with a positive biopsy result, 22 (53.7%) and 19 (46.3%) had a unilaterally and bilaterally positive finding, respectively. Glucocorticoid therapy did not significantly affect the findings of quadriceps femoris muscle biopsies. Unilateral biopsies may have a lower diagnostic sensitivity for AAV than bilateral biopsies. Key Points • Glucocorticoid therapy does not significantly impact the results of quadriceps femoris muscle biopsies in patients with antineutrophil cytoplas¬mic antibody (ANCA)-associated vasculitis (AAV). • Unilateral biopsies may have a lower diagnostic sensitivity for AAV than bilateral biopsies. [ABSTRACT FROM AUTHOR]

Background: There may be some diversity in the practice of co-prescribing 2-mercaptoethane sodium sulfonate (mesna) with cyclophosphamide (CYC) for ANCA-associated vasculitis (AAV). Objectives: To assess the practice of prescribing mesna prophylaxis for CYC-treated patients with AAV. Methods: We invited authors of publications on AAV referenced in MEDLINE over the previous 10 years to participate in an anonymous online survey. Respondents were eligible if they were involved in CYC treatments for AAV. The survey asked about the characteristics of the respondents and their practice in using CYC and mesna to treat AAV and the underlying rationale. We compared 18 variables between mesna prescribers and their counterparts to identify factors associated with mesna use. Results: In total, 139 eligible individuals completed the survey. The participants were from 34 countries and were essentially physicians (98%). Overall, 68%, 19% and 13% of respondents prescribed mesna systematically, never, or on a selective basis. As compared with never/selective-prescribers, systematic-prescribers were more often ≤ 39 years old (P = 0.008), more often used intermittent pulse therapy as the exclusive/predominant CYC administration scheme (P < 0.001), were more frequently based in France/Germany/Italy than in England/United States (P < 0.001), and more often indicated adherence to local standards (P = 0.003) or (inter)national guidelines for AAV (P < 0.001) as a rationale for their mesna practice. Never/selective-prescribers more commonly reported that their mesna prescription pattern had changed as compared with their former practice (P < 0.001). Conclusions: Systematic co-prescription of mesna is the prevailing practice for CYC treatments for AAV. The practice seems to involve practicability considerations and differs between generations. [ABSTRACT FROM AUTHOR]

Rituximab (RTX) has been reported to effectively maintain remission in anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). In this multicenter study involving 57 patients who achieved remission after 24 weeks, we evaluated the effectiveness of RTX in maintaining remission in patients with AAV. Patients were divided into three groups based on RTX administration: continuous, induction phase-only, and maintenance phase-only groups. The continuous group had a remission maintenance rate after 48 weeks of treatment compared with the induction phase-only group (100% vs. 88.2%, p = 0.29). More patients in the continuous group received three or more RTX doses during the induction period (82.4% vs. 52.9%, p = 0.06), and this group had a lower incidence of infection (5.9% vs. 29.4%, p = 0.08). Compared with the maintenance-only group, the continuous group had a numerically higher proportion of patients in remission after 48 weeks of treatment (100% vs. 83.3%, p = 0.26) and a lower incidence of infection (5.9% vs. 50%, p = 0.04); however, the N in the maintenance phase was small and suspected to have low power. Regardless of the method of RTX administration (induction phase-only or continuous), administering RTX during the induction phase may be crucial for achieving remission.

OBJECTIVE: This study examined the relationship between age at diagnosis and disease characteristics and damage in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: Analysis of a prospective longitudinal cohort of patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic GPA (EGPA) in the Vasculitis Clinical Research Consortium (2013-2021). Disease cohorts were divided by age at diagnosis (years): children (65). Data included demographics, ANCA type, clinical characteristics, Vasculitis Damage Index (VDI) scores, ANCA Vasculitis Index of Damage (AVID) scores, and novel disease-specific and non-disease-specific damage scores built from VDI and AVID items. RESULTS: Analysis included data from 1020 patients with GPA/MPA and 357 with EGPA. Female predominance in GPA/MPA decreased with age at diagnosis. AAV in childhood was more often GPA and proteinase 3-ANCA positive. Children with GPA/MPA experienced more subglottic stenosis and alveolar hemorrhage; children and young adults with EGPA experienced more alveolar hemorrhage, need for intubation, and gastrointestinal involvement. Older adults (GPA/MPA) had more neurologic manifestations. After adjusting for disease duration, medications, tobacco, and ANCA, all damage scores increased with age at diagnosis for GPA/MPA (P

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]

Children with anti-neutrophil cytoplasmic antibody-associated vasculitis benefit immensely from avacopan as it reduces the requirement for steroids. However, descriptions of adverse drug reactions in children are lacking, and the dosage and follow-up intervals are unclear. A 10-year-old boy with initial granulomatosis and polyangiitis presented with diffuse pulmonary hemorrhage. Rituximab and 30 mg avacopan were administered twice daily as induction therapy following methylprednisolone pulse therapy. However, sudden liver function test abnormalities were observed on day 31 of avacopan treatment, despite liver enzyme levels being within the normal range 5 days earlier. A drug-induced lymphocyte stimulation and various infectious disease tests yielded negative results. Discontinuation of rituximab and avacopan resulted in improved liver function; no change in the Birmingham Vasculitis Activity Score during liver function test abnormalities was observed. Avacopan-associated abnormalities in liver function tests suggest that drug-induced liver injury may occur rapidly in children, and appropriate dosing strategies should be reconsidered. [ABSTRACT FROM AUTHOR]

A 67-year-old woman with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis was not vaccinated against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and was on multiple immunosuppressive drugs. She was hospitalized because of interstitial shadowing in the lungs and diagnosed with persistent coronavirus disease 2019 (COVID-19). Despite treatment with a recombinant monoclonal antibody and antivirals, her symptoms persisted and she lacked a specific antibody response. She tested negative for SARS-CoV-2 antigen after the second antiviral treatment, and a subsequent chest radiograph showed improvement. However, the antibody levels did not change. This case highlights the importance of careful monitoring of the SARS-CoV-2 antigen and antibody levels during COVID-19 treatment in patients with immunosuppression. [ABSTRACT FROM AUTHOR]