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. <Table Float="No" ID="Taba"> <tgroup cols="2"> <colspec align="left" colname="c1" colnum="1" /> <colspec align="left" colname="c2" colnum="2" /> <tbody> <row> <entry align="left" nameend="c2" namest="c1"> 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. </entry> </row> </tbody> </tgroup> </Table>

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

期刊论文
发表日期：

2025年4月9日

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Anna Hasegawa,
Kazuhiro Kurasawa,
Ryota Koike,
Rika Sato,
Azusa Kikuchi,
Sara Komatsu,
Yusuke Sakaue,
Yuki Aizawa,
Aya Shimizu,
Yuhi Yoshida,
Tomoka Hiyama,
Tomoyuki Miyao,
Ayae Tanaka,
Satoko Arai,
Reika Maezawa,
Masafumi Arima,
Kei Ikeda

摘要

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.