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Multicenter Study
. 2025 Aug 1;64(8):4546-4554.
doi: 10.1093/rheumatology/keaf172.

Immune checkpoint inhibitors-induced large vessel vasculitis: clinical characteristics and management from a European multicentre study

Adrien Cottu  1 , Laure Delaval  2 , Alexandra Forestier  3 , Alessandro Tomelleri  4 , Corrado Campochiaro  4 , Milena Bond  5 , Jérémie Dion  6 , Aline Gury  7 , Xavier Savary  8 , Robin Dhote  9 , Albrecht Betrains  10 , Laurence Bouillet  11   12 , Eric Liozon  13 , Eva Bories  14 , Arthur Petitdemange  1 , Paul Legendre  15 , Benjamin Crichi  16 , Philippe Kerschen  17 , Lucie Carneiro Esteves  18 , Guillaume Armengol  19 , Roderau Outh  20 , Omar Al Tabaa  21 , Louis Wolff  22 , Maxime Ilzkovitz  22 , Mohammad Yassine Cherif  23 , Ariane Laparra  24 , Lorenzo Dagna  4 , Patrick Bruneval  25 , Benjamin Terrier  1   26
Affiliations
Multicenter Study

Immune checkpoint inhibitors-induced large vessel vasculitis: clinical characteristics and management from a European multicentre study

Adrien Cottu et al. Rheumatology (Oxford). .

Abstract

Objective: Immune checkpoint inhibitors (ICIs) have revolutionized cancer treatment but frequently cause immune-related adverse events (IrAEs). ICI-induced large vessel vasculitis (LVV) is a rare IrAE, with limited available data. We aimed to describe and compare clinical characteristics and evolution of ICI-induced LVV to primary LVV.

Methods: This retrospective, multicentre European study included adult patients who developed LVV after ICI therapy between March 2018 and August 2024. Patients were compared with matched controls with primary LVV. LVV was defined histologically and/or morphologically.

Results: Twenty-seven patients were included [median (interquartile range) age 68 (61-74) years]. Five (19%) had a history of polymyalgia rheumatica or GCA. Median time from ICIs initiation to LVV diagnosis was 3 (2-17) months. Eight patients (30%) received ipilimumab-nivolumab combination therapy, all of whom developed LVV within 6 months. Most clinical features were similar to matched controls, however PET scan diagnosis and large-vessel involvement were more common in ICI-induced LVV. Visual loss occurred in 4 (15%) patients. Management included discontinuation of ICIs in 19 (70%) patients and administration of glucocorticoids. After a median follow-up of 12 (5-20) months, 20 (74%) achieved sustained remission. Relapse or treatment failure of LVV occurred in 3/9 (33%) patients who continued ICIs and 4/18 (22%) who discontinued them. By last follow-up, 9 (33%) patients had died, mainly from cancer (n = 8).

Conclusion: ICI-induced LVV is a rare, early-onset IrAE. Although continuation of ICIs may affect LVV outcomes, cancer progression remains the primary cause of mortality. Prospective studies are warranted to better balance therapeutic approaches.

Keywords: cancer; immunotherapy; vasculitis.

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