Le syndrome des antisynthétases : étude rétrospective à propos d’une série de 14 patients

Abstract

PurposeAntisynthetase 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 methodsFourteen patients with antisynthetase syndrome followed-up between 1997 and 2009 were included. We studied retrospectively their clinical, radiological, and pathological findings. ResultsThe 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. ConclusionThe diffusion of immunologic assay will help us in the future to identify the specificity of this syndrome in order to improve care.