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Objective: Data on ANCA-associated vasculitis (AAV) induced by anti-thyroid drugs (ATD) are scarce. We aimed to describe the characteristics and outcome of these patients in comparison to primary AAV., Methods: We performed a retrospective multicentre study including patients with ATD-induced AAV. We focused on ATD-induced microscopic polyangiitis (MPA) and compared them with primary MPA by matching each case with four controls by gender and year of diagnosis., Results: Forty-five patients with ATD-induced AAV of whom 24 MPA were included. ANCA were positive in 44 patients (98%), including myeloperoxidase (MPO)-ANCA in 21 (47%), proteinase 3 (PR3)-ANCA in six (13%), and double positive MPO- and PR3-ANCA in 15 (33%). Main clinical manifestations were skin involvement (64%), arthralgia (51%) and glomerulonephritis (20%). ATD was discontinued in 98% of cases, allowing vasculitis remission in seven (16%). All the remaining patients achieved remission after glucocorticoids, in combination with rituximab in 11 (30%) or cyclophosphamide in four (11%). ATD were reintroduced in seven cases (16%) without any subsequent relapse. Compared with 96 matched primary MPA, ATD-induced MPA were younger at diagnosis (48 vs 65 years, P < 0.001), had more frequent cutaneous involvement (54 vs 25%, P = 0.007), but less frequent kidney (38 vs 73%, P = 0.02), and a lower risk of relapse (adjusted HR 0.07; 95% CI 0.01, 0.65, P = 0.019)., Conclusion: ATD-induced AAV were mainly MPA with MPO-ANCA, but double MPO- and PR3-ANCA positivity was frequent. The most common manifestations were skin and musculoskeletal manifestations. ATD-induced MPA were less severe and showed a lower risk of relapse than primary MPA., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Updated guidelines on the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) were released in 2021 by the American College of Rheumatology jointly with the Vasculitis Foundation and, subsequently, in 2022 by the European Alliance of Associations for Rheumatology. In addition, in 2021, the Kidney Disease: Improving Global Outcomes had released updated recommendations on the treatment of AAV with glomerulonephritis (AAV-GN). Kidney involvement is particularly relevant in microscopic polyangiitis and granulomatosis with polyangiitis, but is less frequent in eosinophilic granulomatosis with polyangiitis. The management of AAV-GN has been a focus for drug development and change over the past 10 years. Avoidance of progression to end-stage kidney disease (ESKD) or kidney failure is one of the main unmet needs in the management of AAV, with ESKD having a major impact on morbidity, health costs and mortality risk. Relevant changes in AAV-GN management are related to remission-induction treatment of patients with severe kidney disease, the use of glucocorticoids and avacopan, and remission-maintenance treatment. All the documents provide guidance in accordance with the evidence-based standard of care available at the time of their release. With our work we aim to (i) show the progress made and identify the differences between guidelines and recommendations, (ii) discuss the supporting rationale for those, and (iii) identify gaps in knowledge that could benefit from additional research and should be revised in subsequent updates., (© The Author(s) 2023. Published by Oxford University Press on behalf of the ERA.)

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 literature search and to create biomarker panels., 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-18BP) 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-18BP 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-18BP in blood and MCP-1 and C5a in urine samples. Moreover, we propose a biomarker panel comprising CRP and urinary MCP-1 in patients with AAV and renal involvement. Further investigations to confirm our preliminary results are clearly warranted, including the reliability to predict disease relapses., (© The Author 2015. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.)