Clinical features and risk factors of flare in anti-synthetase syndrome

• Published in: Clinical rheumatology Vol. 44; no. 6; pp. 2431 - 2438
• Main Authors: Hasegawa, Anna, Kurasawa, Kazuhiro, Koike, Ryota, Sato, Rika, Kikuchi, Azusa, Komatsu, Sara, Sakaue, Yusuke, Aizawa, Yuki, Shimizu, Aya, Yoshida, Yuhi, Hiyama, Tomoka, Miyao, Tomoyuki, Tanaka, Ayae, Arai, Satoko, Maezawa, Reika, Arima, Masafumi, Ikeda, Kei
• Format: Journal Article
• Language: English
• Published: Cham Springer International Publishing 01.06.2025; Springer Nature B.V
• Subjects: Adult; Aged; Amino Acyl-tRNA Synthetases - immunology; Aminoacyl-tRNA ligase; Antibodies; Antibodies, Antinuclear; Calcineurin inhibitors; Calcineurin Inhibitors - therapeutic use; Female; Glucocorticoids; Glucocorticoids - therapeutic use; Humans; Immunosuppressive Agents - therapeutic use; Lung Diseases, Interstitial - drug therapy; Male; Medical records; Medicine; Medicine & Public Health; Middle Aged; Myositis; Myositis - drug therapy; Myositis - immunology; Original Article; Patients; Prednisolone; Prednisolone - therapeutic use; Retrospective Studies; Rheumatology; Risk Factors; tRNA; Myositis; Flare; Anti-aminoacyl-tRNA synthetase antibody; Interstitial lung disease; Anti-synthetase syndrome
• ISSN: 0770-3198; 1434-9949; 1434-9949
• DOI: 10.1007/s10067-025-07398-9

Objectives To clarify clinical features and risk factors of flares in anti-synthetase syndrome (ASS). Methods We retrospectively identified consecutive patients with ASS by reviewing medical records. Clinical, laboratory, and imaging data were obtained. Patients were considered to have a flare when treatment was intensified for myositis and/or ILD with an increased dose of glucocorticoid (GC) and/or addition of a new immunosuppressant (IS). Results A total of 51 subjects were included. All 51 patients had ILD, 38 patients had myositis, and 39 patients had skin rash. Anti-aminoacyl-tRNA synthetase (ARS) antibody subtypes were anti-Jo-1 in 23 (45%), anti-EJ in 13 (25%), anti-PL-7 in 8 (16%), and anti-PL-12 in 7 (14%). As initial therapy, half of the patients were treated with GCs alone, while the rest were treated with a combination of GCs and ISs during observation. Flares occurred in 30 (58%) mostly when the prednisolone dose was tapered to 11 mg/day. Patients with anti-EJ or anti-Jo-1 frequently flared. The use of calcineurin inhibitors (CNIs) was significantly associated with less flares of myositis but not with those of ILD. In multivariate analyses, anti-ARS subtype and Gottron’s sign were identified as independent risk factors for overall flare. Conclusion The majority of ASS patients experienced a flare, which frequently occurred when the GC dose was tapered to 11 mg of prednisolone. The presence of anti-EJ/Jo-1 antibody and Gottron’s sign were identified as independent risk factors for flare. In addition, the use of CNIs tended to associate with less flares of myositis. Key Points • The majority of patients with anti-synthetase syndrome experience a flare when glucocorticoids are tapered close to 10 mg of prednisolone . • The presence of anti-EJ/Jo-1 antibody and Gottron’s sign are risk factors for flares in patients with anti-synthetase syndrome . • The use of calcineurin inhibitors may prevent flares of myositis in patients with anti-synthetase syndrome .