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The Diagnosis and Treatment of Antisynthetase Syndrome

The Diagnosis and Treatment of Antisynthetase Syndrome 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. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Clinical Pulmonary Medicine Wolters Kluwer Health

The Diagnosis and Treatment of Antisynthetase Syndrome

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References (74)

Publisher
Wolters Kluwer Health
Copyright
Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved.
ISSN
1068-0640
eISSN
1536-5956
DOI
10.1097/CPM.0000000000000171
Publisher site
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Abstract

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.

Journal

Clinical Pulmonary Medicine – Wolters Kluwer Health

Published: Sep 1, 2016

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