Clinical associations of renal involvement in ANCA-associated vasculitis

Andreas Kronbichler, Jae Il Shin, Keum Hwa Lee, Daiki Nakagomi, Luis F. Quintana, Martin Busch, Anthea Craven, Raashid A. Luqmani, Peter A. Merkel, Gert Mayer, David R.W. Jayne, Richard A. Watts

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28 Citations (Scopus)


Objective: Renal involvement in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis is associated with significant morbidity and higher mortality rates. This study examined clinical manifestations associated with renal involvement in ANCA-associated vasculitis within a large, international cross-sectional cohort. Methods: Univariate and multivariate analyses were performed to identify clinical factors associated with renal disease, which was defined as i) a serum-creatinine >30% above normal and a fall in creatinine-clearance >25%; or ii) haematuria attributable to active vasculitis. Results: The study cohort include 1230 patients from 31 countries; 723 (58.8%) presented with renal involvement: microscopic polyangiitis (82.2%), granulomatosis with polyangiitis (58.6%), and eosinophilic granulomatosis with polyangiitis (26.4%). The following clinical and laboratory factors were more common among patients with renal disease: age (OR 1.01, 95% CI 1.01–1.02), fever (OR 1.97, 95% CI 1.35–2.88), fatigue (OR 1.55, 95% CI 1.14–2.10), weight loss (OR 1.62, 95% CI 1.23–2.12), polyarthritis (OR 1.39, 95% CI 1.02–1.89), petechiae/purpura (OR 1.47, 95% CI 1.06–2.05), pulmonary haemorrhage (OR 5.23, 95% CI 1.39–19.63), gastrointestinal symptoms (OR 2.19, 95% CI 1.34–3.58), seizures (OR 3.42, 95% CI 1.26–9.30), lower serum albumin (OR 2.42, 95% CI 1.64–3.57), higher CRP (OR 2.06, 95% CI 1.04–4.06), low serum C3 at baseline (OR 3.86, 95% CI 1.30–11.53), myeloperoxidase- (OR 7.97, 95% CI 2.74–23.20) and proteinase 3-ANCA (OR 3.40, 95% CI 1.22–9.50). The following clinical factors were less common among patients with renal disease: mononeuritis multiplex (OR 0.63, 95% CI 0.41–0.98), proptosis/exophthalmos (OR 0.19, 95% CI 0.06–0.59), nasal polyps (OR 0.32, 95% CI 0.19–0.55), septal defect/perforation (OR 0.29, 95% CI 0.14–0.60), respiratory distress/pulmonary fibrosis/asthma (OR 0.08, 95% CI 0.04–0.19), and wheeze/obstructive airway disease (OR 0.29, 95% CI 0.16–0.52). Conclusion: In this large international study, several clinical and laboratory factors were identified as associated with renal involvement in ANCA-associated vasculitis.

Original languageEnglish
Article number102495
JournalAutoimmunity Reviews
Issue number4
Publication statusPublished - 2020 Apr

Bibliographical note

Funding Information:
Dr. Kronbichler received consulting fees from Vifor Pharma and speaking fees from Novartis, TerumoBCT and Miltenyi Biotech. Dr. Luqmani received research support from Roche, Vifor Pharma and InflaRx and speaking fees from Roche. Dr. Merkel received research support from AstraZeneca, Boehringer-Ingelheim, Bristol-Myers Squibb, Celgene, ChemoCentryx, Genentech/Roche, Genzyme/Sanofi, GlaxoSmithKline, Kypha and TerumoBCT, consulting fees from Abbvie, AstraZeneca, Biogen, Boehringer-Ingelheim, Bristol-Myers Squibb, Celgene, ChemoCentryx, CSL Behring, Genentech/Roche, Genzyme/Sanofi, GlaxoSmithKline, InflaRx, Insmed, Jannsen, Kiniksa, royalties from UpToDate. Dr. Jayne received received research grants from ChemoCentryx, GlaxoSmithKline, Genentech/Roche and Genzyme/Sanofi, and consulting fees from AstraZeneca, Boehringer-Ingelheim, Celgene, Insmed and Takeda. All potential conflicts of interest are outside the submitted work. All other authors report no potential conflicts.

Publisher Copyright:
© 2020 Elsevier B.V.

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Immunology


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