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The Diagnosis and Treatment of Antisynthetase Syndrome

Witt, Leah J. MD; Curran, James J. MD; Strek, Mary E. MD

doi: 10.1097/CPM.0000000000000171
Interstitial, Inflammatory & Occupational Lung Disease

Antisynthetase syndrome is an autoimmune condition, characterized by antibodies directed against an aminoacycl transfer RNA synthetase along with clinical features that can include interstitial lung disease (ILD), myositis, Raynaud’s phenomenon, and arthritis. There is a higher prevalence and increased severity of ILD in patients with antisynthetase syndrome, as compared with dermatomyositis and polymyositis, inflammatory myopathies with which it may overlap phenotypically. The diagnosis is made by a multidisciplinary approach, synthesizing rheumatology and pulmonary evaluations, along with serologic, radiographic, and occasionally muscle and/or lung biopsy results. Patients with antisynthetase syndrome often require multimodality immunosuppressive therapy to control the muscle and/or pulmonary manifestations of their disease. The long-term care of these patients mandates careful attention to the adverse effects and complications of chronic immunosuppressive therapy, and disease-related sequelae that can include progressive ILD necessitating lung transplantation, pulmonary hypertension, malignancy, and a decreased survival. It is expected that a greater awareness of the clinical features of this syndrome will allow for an earlier diagnosis and appropriate treatment to improve outcomes in patients with antisynthetase syndrome.

*Section of Pulmonary and Critical Care Medicine, Department of Medicine

Section of Rheumatology, Department of Medicine, The University of Chicago Medicine, Chicago, IL

Supported by the NIH funded Research Training in Respiratory Biology grant at the University of Chicago (T32 HL007605).

Disclosure: The authors declare that they have no conflicts of interest.

Address correspondence to: Leah J. Witt, MD, The University of Chicago Medicine, 5841 South Maryland Ave., MC 6076, Chicago, IL 60637. E-mail:

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