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Antisynthetase syndrome pathogenesis: knowledge and uncertainties

Gallay, Laurea,b; Gayed, Catherinec; Hervier, Baptistec,d

Current Opinion in Rheumatology: November 2018 - Volume 30 - Issue 6 - p 664–673
doi: 10.1097/BOR.0000000000000555
MYOSITIS AND MYOPATHIES: Edited by Andrea Doria and Anna Ghirardello

Purpose of review Antisynthetase syndrome (ASyS) is an acquired myopathy characterized by the presence of myositis-specific autoantibodies directed against tRNA-synthetases. ASyS is potentially life threatening due to lung involvement and treatment remains a challenge to date. With symptoms not limited to muscles but also involving lung, skin and joints, ASyS appears specific and has a particular pathogenesis, different from the other inflammatory myopathies. This review is intended to discuss the current understanding of ASyS pathogenesis, pointing its current knowledge and also the crucial prospects that may lead to critical improvement of ASyS care.

Recent findings Regarding ASyS pathogenesis, initiation of the disease seems to arise in a multifactorial context, with first lesions occurring within the lungs. This may lead to aberrant self-antigen exposure and tolerance breakdown. The consequences are abnormal activation of both innate and adaptive immunity, resulting in the patients with favourable genetic background to autoimmune-mediated organ lesions. Immune and nonimmune roles of the antigen, as well as antigen presentation leading to specific T-cell and B-cell activation and to the production of specific autoantibodies belong to the disease process.

Summary This work aims to detail ASyS pathogenesis understanding, from initiation to the disease propagation and target tissue lesions, in order to considering future treatment directions.

aDépartement de Médecine Interne et Immunologie Clinique, Centre Hospitalo-Universitaire Edouard Herriot, Hospices Civils de Lyon

bINMG, CNRS UMR 5310 - INSERM U1217, University Claude Bernard, Lyon 1

cSorbonne Universités, Université Pierre et Marie Curie Université Paris 06, INSERM U1135, CNRS ERL8255, Centre d’Immunologie et des Maladies Infectieuses

dDépartement de Médecine Interne et Immunologie Clinique, Centre National de Référence des Maladies Musculaires, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France

Correspondence to Baptiste Hervier, Département de Médecine Interne et Immunologie Clinique, Hôpital de la Pitié-Salpêtrière, 47–83 boulevard de l’Hôpital, 75651 Paris cedex 13, France. Tel: +33142161056; fax: +33142161065; e-mail: baptiste.hervier@aphp.fr

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