ANCA-associated scleritis: impact of ANCA on presentation, response to therapy and outcome

Autor: Laura Perray, Yann Nguyen, Gaëlle Clavel Refregiers, Thibaud Chazal, Emmanuel Héron, Clara Pouchelon, Bertrand Dunogué, Nathalie Costedoat-Chalumeau, Anne Murarasu, Alexis Régent, Xavier Puéchal, Benjamin Thoreau, François Lifermann, Julie Graveleau, Miguel Hié, Antoine Froissart, Antoine Baudet, Alban Deroux, Christian Lavigne, Sébastien Puigrenier, Rafik Mesbah, Thomas Moulinet, Claire Vasco, Sabine Revuz, Grégory Pugnet, Virginie Rieu, Anaïs Combes, Antoine Brézin, Benjamin Terrier
Rok vydání: 2023
Předmět:
Zdroj: Rheumatology.
ISSN: 1462-0332
1462-0324
Popis: Objectives To describe characteristics, treatment, and outcome of isolated antineutrophil cytoplasm antibody (ANCA)-associated scleritis at diagnosis, in comparison with idiopathic scleritis with negative ANCA tests. Methods This retrospective case control multicentre study was performed within the French Vasculitis Study Group (FVSG) network and in three French tertiary ophthalmologic centres. Data of patients with scleritis without any systemic manifestation and with positive ANCA results were compared to those of a control group of patients with idiopathic scleritis with negative ANCA tests. Results 120 patients, including 38 patients with ANCA-associated scleritis and 82 control patients, diagnosed between January 2007 and April 2022, were included. Median follow-up was 28 months (IQR 10–60). Median age at diagnosis was 48 (IQR 33–60) and 75% were female subjects. Scleromalacia was more frequent in ANCA-positive patients (p = 0.027). 54% had associated ophthalmologic manifestations, without significant differences. ANCA-associated scleritis more frequently required systemic medications, including glucocorticoids (76% versus 34%, p 5 mg/L at diagnosis was the only significant risk factor of progression to systemic AAV (adjusted HR 5.85; 95%CI 1.10–31.01; p = 0.038). Conclusion Isolated ANCA-associated scleritis are mostly anterior scleritis with higher risk of scleromalacia than ANCA-negative idiopathic scleritis and are more often difficult-to-treat. One third of patients with PR3- or MPO-ANCA scleritis progressed to systemic AAV.
Databáze: OpenAIRE
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