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The Diagnosis and Treatment of Antisynthetase Syndrome
#MMPMID27594777
Witt LJ
; Curran JJ
; Strek ME
Clin Pulm Med
2016[Sep]; 23
(5
): 218-226
PMID27594777
show ga
Anti-synthetase 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, myositis, Raynaud's
phenomenon, and arthritis. There is a higher prevalence and increased severity of
interstitial lung disease in patients with anti-synthetase syndrome, as compared
to dermatomyositis and polymyositis, inflammatory myopathies with which it may
overlap phenotypically. 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
anti-synthetase syndrome often require multi-modality immunosuppressive therapy
in order 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, as well as
disease-related sequelae that can include progressive interstitial lung disease
necessitating lung transplantation, pulmonary hypertension, malignancy and
decreased survival. It is hoped that greater awareness of the clinical features
of this syndrome will allow for earlier diagnosis and appropriate treatment to
improve outcomes in patients with anti-synthetase syndrome.
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