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Critical Reviews™ in Immunology
Factor de Impacto: 1.352 Factor de Impacto de 5 años: 3.347 SJR: 0.657 SNIP: 0.55 CiteScore™: 2.19

ISSN Imprimir: 1040-8401
ISSN En Línea: 2162-6472

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Critical Reviews™ in Immunology

DOI: 10.1615/CritRevImmunol.2018025744
pages 263-278

Immunopathogenesis of the Anti-Synthetase Syndrome

Catherine Gayed
Sorbonne Université, Université Pierre et Marie Curie, Paris 06, INSERM U1135, Centre National de la Recherche Scientifique, ERL8255, Centre d'Immunologie et des Maladies Infectieuses, Paris, France
Yurdagül Uzunhan
Department of Pulmonology, Assistance Publique-Hopitaux de Paris, Avicenne Hospital, Paris 13 University, Sorbonne Paris Cité, Faculty of Medicine Santé, Médecine, Biologie Humaine, Bobigny, France
Isabelle Cremer
INSERM Unité Mixte de Recherche Scientifique 1138, Centre de Recherche des Cordeliers, Université Pierre et Marie Curie, Paris, France
Vincent Vieillard
Sorbonne Université, Université Pierre et Marie Curie, Paris 06, INSERM U1135, Centre National de la Recherche Scientifique, ERL8255, Centre d'Immunologie et des Maladies Infectieuses, Paris, France
Baptiste Hervier
Sorbonne Université, Université Pierre et Marie Curie, Paris 06, INSERM U1135, Centre National de la Recherche Scientifique, ERL8255, Centre d'Immunologie et des Maladies Infectieuses, Paris, France; Service de Médecine Interne et immunologie Clinique, Centre National de Référence des Maladies Neuromusculaires Rares, Hôpital Pitié-Salpêtrière, Assistance Publique-Hopitaux de Paris, Paris, France

SINOPSIS

Among the inflammatory myopathies, anti-tRNA-synthetase syndrome (ASyS) is a severe autoimmune condition characterized by extramuscular involvement, affecting especially the lungs. ASyS specific serological markers are anti-tRNA-synthetase autoantibodies, among which anti-histidyl-tRNA-synthetase is the most common. In the past decades, ASyS has been distinguished by unique histological features attributed to a specific pathogenesis. Research has highlighted the role of environmental factors and infections as possible triggers. Tissue modifications of histidyl-tRNA-synthetase (HisRS) expression might be responsible for the recruitment and activation of both innate and adaptive immune cells. HisRS not only acts through antigenic properties, but also through many others, including chemoattraction, innate pathway activation, and cytokine-like functions. Favored by a certain genetic background, this whole activation of immunity results in widespread and specific tissue damage and finally leads to visible heterogeneous symptoms characterizing the disease state. Understanding the pathogenesis of ASyS is essential to improving patient care by identifying biomarkers and designing new therapeutic strategies accordingly. Therefore, this review details the recent hypotheses concerning the dynamic of ASyS pathogenesis with the aim of enlightening the development of new therapeutic axes in the future.