ORIGINAL RESEARCH article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1506457
This article is part of the Research TopicAutoimmunity: novel insights and future perspectivesView all 40 articles
Vasculitis as an Indicator of Disease Severity in Familial Mediterranean Fever
Provisionally accepted
- 1dermatology department, Sheba Medical Center, Ramat Gan, Israel
- 2School of Medicine, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
- 3The Danek Gertner Institute of Human Genetics, Sheba Medical Center, Tel Hashomer, Israel
Select one of your emails
You have multiple emails registered with Frontiers:
Notify me on publication
Please enter your email address:
If you already have an account, please login
You don't have a Frontiers account ? You can register here
Introduction: Various types of vasculitides have been identified in patients with familial Mediterranean fever (FMF); however, FMF characteristic in patients who experience vasculitis during the disease course have not been described. This study aimed to describe the types of vasculitides in FMF and characterize the patients. Methods: This nested case-control study compared 27 patients with FMF (12 male) diagnosed with vasculitis with 100 patients (49 men) who did not develop vasculitis. Results: Most patients (25/27) developed vasculitis after FMF diagnosis. Four types of vasculitides were observed: cutaneous small vessel vasculitis (10 patients, 37%), Henoch-Schonlein purpura/immunoglobulin A vasculitis (8 patients, 30%), periarteritis nodosa (three patients, 11%), and Behçet disease (six patients, 22%). The vasculitis group was younger at FMF onset (6.6 [± 5.9] years vs. 16.2 [± 13.7] years, p < 0.002) and diagnosis (13.1 [± 13.1] years vs. 25.1 [± 17.92] years, p < 0.001). This group showed a higher frequency of homozygosity for the M694V mutation (73.9% vs. 29.4%, p < 0.001), had a more severe FMF (mean Pras severity score: 10.4 [± 2.6] vs. 7.3 [± 3.1], p < 0.001), required higher colchicine doses (1.96 [± 0.61] mg/d vs. 1.66 [± 0.65] mg/d, p < 0.025), and tended to show higher rates of colchicine resistance (29.6% vs. 12%, p = 0.053). However, vasculitis was not an independent factor influencing FMF severity. Conclusion: Patients with FMF and vasculitis are characterized by a more severe disease, likely due to factors other than vasculitis itself. Yet, its presence can serve as a clinical clue to disease severity.
Keywords: Familial Mediterranean Fever, Vasculitis, superficial cutaneous vasculitis, immunoglobulin vasculitis/Henoch-Schonlein purpura, Polyarteritis Nodosa, Behçet disease, Pras severity score
Received: 05 Oct 2024; Accepted: 10 Jul 2025.
Copyright: © 2025 Barzilai, Mash, Gershon, Pras, Baum and Pras. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Aviv Barzilai, dermatology department, Sheba Medical Center, Ramat Gan, Israel
Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.