Antisynthetase syndrome: A retrospective study of 14 patients

作者：Dieval C^*; Ribeiro E; Mercie P; Blanco P; Duffau P; Longy Boursier M

来源：Revue de Medecine Interne, 2012, 33(2): 76-79.

DOI：10.1016/j.revmed.2011.11.017

• Anti-aminoacyl-t-RNA synthetase autoantibodies
• Myositis
• Intersitial lung disease

Purpose. - Antisynthetase syndrome is a rare entity characterized by myositis (dermatomyositis or polymyositis), interstitial lung disease, arthritis, Raynaud's phenomena and mechanic's hands skin manifestation, and the presence of autoantibodies against aminoacyl-transfer RNA synthetase.
Patients and methods. - Fourteen patients with antisynthetase syndrome followed-up between 1997 and 2009 were included. We studied retrospectively their clinical, radiological, and pathological findings.
Results. - The sex ratio women/men was 2.5. Mean age at disease onset was 46 years. Arthritis (43%) and interstitial lung disease (38%) were the most frequent features at disease onset. Seven patients had myositis. Ten patients had anti-Jo1 autoantibodies, three had anti-PL7 and one anti-PL12. Corticosteroid therapy was given in all cases, immunosuppressive drugs in 12 cases, due to initial severity (n = 8), disease relapse (n= 3) or,corticosteroid dependence (n=1). After a mean follow-up of 64 months, nine patients improved, four stabilized and one patient died after lung transplantation, required for pulmonary hypertension.
Conclusion. - The diffusion of immunologic assay will help us in the future to identify the specificity of this syndrome in order to improve care.

• 出版日期2012-2