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Multicenter Study
. 2025 Jun 1;64(6):3685-3690.
doi: 10.1093/rheumatology/keae711.

Immune checkpoint inhibitors-associated vasculitis: a heterogeneous condition with possible severe disease course

Noemie Chanson  1 , Alexandre Galvagni  1 , Manuel Ramos-Casals  2   3   4 , Juan Ignacio Ruiz  5 , Karijn P M Suijkerbuijk  6 , Karolina Gente  7 , Philippe Kerschen  8 , Jean Denis Karam  9 , Rakiba Belkhir  10 , Rodereau Outh  11 , Fabienne Closs-Prophette  12 , Jose Salvador Garcia Morillo  13 , Ángel Robles-Marhuenda  14 , Jean-Marie Michot  15 , Anne Laure Voisin  15 , Sabine Messayke  15 , Arianne Laparra  16 , Caroline Robert  17 , Maria Suarez-Almazor  5 , Xavier Mariette  10   18 , Olivier Lambotte  1   18 ; ICIR
Collaborators, Affiliations
Multicenter Study

Immune checkpoint inhibitors-associated vasculitis: a heterogeneous condition with possible severe disease course

Noemie Chanson et al. Rheumatology (Oxford). .

Abstract

Objective: To describe presentation, treatment and outcome of immune checkpoint inhibitor (ICI) associated-vasculitis in cancer patients in a multicentre study.

Methods: Thanks to the ImmunoCancer International Registry (ICIR), a multidisciplinary network focused on the research of the immune related adverse events related to cancer immunotherapies, patients presenting with a clinical and/or radiological suspicion of vasculitis and histological evidence of vasculitis after being exposed to ICIs were retrospectively identified.

Results: Twenty-eight cases were identified in the ICIR registry. The median interval between starting ICI treatment and vasculitis diagnosis was 4 months. Small vessel vasculitis was predominant (n = 21), followed by large vessel (n = 4) and medium vessel (n = 3). The small vessel vasculitis included 10 unclassified vasculitis either with limited cutaneous involvement (n = 6) or systemic involvement (n = 4), five IgA vasculitis, three cryoglobulinemic vasculitis, and three ANCA+ vasculitis. At presentation or during the evolution, renal and neurologic manifestations were evidenced in seven cases each (25%). Renal biopsies documented immune glomerulopathies in six cases. Only seven patients (25%) fulfilled the 2022 ACR/EULAR classification criteria (four giant cell arteritis, two EGPA and one GPA). Most patients (90%) required systemic corticosteroid and an additional drug was given in 10 patients (36%). Vasculitis outcome was good: 22 patients had vasculitis complete response, no patient died due to vasculitis. Nine patients (32%) were rechallenged with immunotherapy with only one relapse.

Conclusion: ICI-associated vasculitis are rare, heterogeneous, but can be severe requiring urgent multidisciplinary management with aggressive treatment.

Keywords: aortitis; cancer immunotherapy; glomerulonephritis; immune checkpoint inhibitor; immune-related adverse events; large vessels vasculitis; purpura; small vessels vasculitis; vasculitis.

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