IgG4-related uveitis. A French cohort and literature review.

Autor: Stuchfield-Denby E; Médecine interne, CHU Gabriel Montpied, Clermont-Ferrand, France. Electronic address: emily.stuchfield-denby@doctorant.uca.fr., De Sainte Marie B; Médecine interne, Hôpital de la Timone, AP-HM, Aix-Marseille Université, Marseille, France., Hie M; Medecine interne, Hôpital Pitié-Salpêtrière, Paris, France., Hatchuel Y; Pédiatrie, Hôpital Pierre Zobda-Quitman, CHU de Martinique, Fort de France, Martinique., Gerber A; Médecine interne, CHU Nord Félix Guyon, Saint-Denis, La Réunion, France., Bencheikh S; Médecine interne, Centre Hospitalier de Perpignan, Perpignan, France., Pugnet G; Service de Médecine Interne et Immunologie Clinique, CHU Rangueil, Toulouse, France., Groh M; National Reference Center for Hypereosinophilic Syndromes (CEREO), Department of Internal Medicine, Foch Hospital, Suresnes, France, Univ. Lille, INSERM U1286 - INFINITE - Institute for Translational Research in Inflammation, Lille, France., Farhat MM; University Lille, Inserm, CHU de Lille, Service de Médecine Interne et Immunologie Clinique, Centre de référence des maladies autoimmunes systémiques rares du Nord et Nord-Ouest de France (CeRAINO), U1286 - INFINITE - Institute for Translational Research in Inflammation, 59000 Lille, France., Urbina D; Service de médecine infantile, Hôpital Timone Enfants, Marseille, France., Ebbo M; Médecine interne, Hôpital de la Timone, AP-HM, Aix-Marseille Université, Marseille, France., Schleinitz N; Médecine interne, Hôpital de la Timone, AP-HM, Aix-Marseille Université, Marseille, France.
Jazyk: angličtina
Zdroj: Seminars in arthritis and rheumatism [Semin Arthritis Rheum] 2024 Feb; Vol. 64, pp. 152278. Date of Electronic Publication: 2023 Nov 11.
DOI: 10.1016/j.semarthrit.2023.152278
Abstrakt: Introduction: IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder that can affect almost any organ. IgG4-related ophthalmic disease is a protean condition involving the orbit and ocular adnexa. Although a few cases of uveitis have been reported, the exact pattern of IgG4-related intraocular manifestations remains unclear. Here, we report on a nationwide French multicenter cohort of patients with IgG4-RD and uveitis and conducted a literature review.
Methods: Patients with uveitis and a concomitant definite diagnosis of IgG4-RD (Revised Comprehensive Diagnostic criteria, American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-RD, International Consensus Diagnostic Criteria for auto-immune pancreatitis, or diagnostic criteria for IgG4-related hypophysitis), were screened from our national IgG4-RD and systemic fibrosis database. Concomitantly, we conducted a PubMed literature review and selected cases of definite IgG4-RD with uveitis.
Results: We reported on 16 patients (8 from our database and 8 from the literature) and a total of 30 episodes of uveitis. Uveitis cases represented 3 % of total IgG4-RD patients in the national database on IgG4-RD and systemic fibrosis. Uveitis was inaugural in IgG4-RD in 4/16 cases (25 %) (appearing before any other IgG4-related symptom, at a median of 9 months), occurred concurrently to other IgG4-related symptoms in 9/16 cases (56 %) (at a median of 15 months before IgG4-RD diagnosis), and appeared during follow up in 3/16 patients (19 %) (at a median of 57 months after first IgG4-related symptoms). When uveitis occurred during follow up, it was associated with IgG4-RD manifestations in other organs in 6/9 patients (67 %). Uveitis was bilateral in 8/16 cases (50 %) and granulomatous in 5/10 cases (50 %). It was anterior in 8/13 (62 %), intermediate in 3/13 (23 %), and global (panuveitis) in 4/13 patients (31 %). Median serum IgG4 at diagnosis was 3.2 g/L. Median follow up time was of 6 years, during which 8/16 patients (50 %) experienced at least one relapse of uveitis. Treatment data was available for 29/30 uveitis flares. Steroids were used in 28/29 episodes of uveitis (97 %), leading to remission of uveitis in 16/28 cases (57 %). Methotrexate and rituximab (in combination with systemic steroids) were administered as second- or third-line therapy in 6/29 (21 %) and 5/29 (17 %) episodes of uveitis, respectively, and led to remission of uveitis in 4/6 cases (67 %) and 4/5 cases (80 %), respectively. One third of uveitides required at least two different lines of treatment for remission induction (mainly a combination of both systemic steroids and methotrexate or rituximab).
Discussion and Conclusions: Uveitis may be one of the initial symptoms of IgG4-RD, and IgG4-RD should be considered in the diagnostic workup of uveitis. Its early onset in IgG4-RD may help with early diagnosis and treatment of the disease. Steroid monotherapy may be sufficient to treat IgG4-related uveitis, yet relapses were frequent (50 %) and ultimately a third of patients required at least two lines of treatment. Hence, steroid-sparing agents can be considered at early stages of the disease, particularly for patients with a high risk of relapse or steroid-related complications.
Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
(Copyright © 2023. Published by Elsevier Inc.)
Databáze: MEDLINE
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