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Pulmonary involvement in antisynthetase syndrome

Gasparotto, Michela; Gatto, Mariele; Saccon, Francesca; Ghirardello, Anna; Iaccarino, Luca; Doria, Andrea

Current Opinion in Rheumatology: November 2019 - Volume 31 - Issue 6 - p 603–610
doi: 10.1097/BOR.0000000000000663
MYOSITIS AND MYOPATHIES: Edited by Andrea Doria, Anna Ghirardello, and Mariele Gatto

Purpose of review Lung involvement is a distinctive feature of antisynthetase syndrome (ASS) and it is considered a basic disease-classifying criterion. In this review, we go over clinical features, radiological patterns, prognostic factors, pathogenesis and treatment of lung involvement in ASS patients, focusing on the clinical differences linked to the different antibody specificities known so far.

Recent findings The lung is the most common extramuscular organ involved in ASS and has the greatest impact on patient prognosis. The pulmonary disease-defining manifestation in ASS is interstitial lung disease (ILD), yet a proportion of patients also develop pulmonary arterial hypertension and, less frequently, obstructive bronchiolitis or acute respiratory failure according to drivers not yet fully understood but likely associated with the underlying autoantibody pattern. Clinical presentation of pulmonary involvement can range from milder forms to a rapidly progressive disease which may lead to chronic lung damage if misdiagnosed and not properly treated.

Summary The knowledge of risk factors associated with progressive or refractory lung damage is important to identify and properly treat patients with the poorest prognosis. For those with a disease not responsive to conventional therapy the efficacy of other therapeutic option is under evaluation.

Division of Rheumatology, Department of Medicine, University of Padua, Padua, Italy

Correspondence to Andrea Doria, Division of Rheumatology, Department of Medicine, University of Padua, Via Giustiniani, 2, 35128 Padua, Italy. Tel: +39 049 8212190; fax: +39 049 8212191; e-mail:

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