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

Rheumatology (Oxford)

December 2024

AP-HP, Université Paris Saclay, department of internal medicine and clinical immunology, Bicêtre Hospital, Le Kremlin Bicêtre, France.

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.

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Polyarteritis nodosa (PAN) is a rare systemic necrotizing vasculitis preferentially targeting medium-sized arteries. PAN has two clinical entities: systemic PAN (sPAN) and cutaneous PAN (cPAN). cPAN is a skin-limited vasculitis, while ulcerative cPAN often predicts a higher risk of recurrence and a worse prognosis.

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Article Synopsis
  • The study aimed to assess the prevalence and characteristics of vasculitis in patients diagnosed with VEXAS syndrome, confirmed through UBA1 mutation.
  • A total of 89 male patients with an average age of nearly 67 years were evaluated, with 23.6% showing signs of vasculitis, predominantly small vessel types.
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