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Interstitial lung disease in dermatomyositis and polymyositis: Treatment

Paul F Dellaripa, MD
Marc L Miller, MD
Section Editors
Ira N Targoff, MD
Kevin R Flaherty, MD, MS
Deputy Editors
Helen Hollingsworth, MD
Monica Ramirez Curtis, MD, MPH


Interstitial lung disease (ILD) is a major cause of morbidity and mortality in dermatomyositis (DM) and polymyositis (PM). The clinical features and histopathologic appearance of ILD in DM and PM reflect the patterns of lung pathology associated with the idiopathic interstitial pneumonias, including nonspecific interstitial pneumonia, usual interstitial pneumonia, organizing pneumonia, and acute interstitial pneumonia. (See "Idiopathic interstitial pneumonias: Clinical manifestations and pathology".)

The treatment of interstitial lung disease associated with DM and PM will be reviewed here. The clinical manifestations, diagnosis, and differential diagnosis of interstitial lung disease in DM and PM and the management of myositis in DM and PM are discussed separately. (See "Interstitial lung disease in dermatomyositis and polymyositis: Clinical manifestations and diagnosis" and "Initial treatment of dermatomyositis and polymyositis in adults" and "Treatment of recurrent and resistant dermatomyositis and polymyositis in adults" and "Juvenile dermatomyositis and polymyositis: Treatment, complications, and prognosis".)


When assessing the need for treatment in patients with interstitial lung disease (ILD) due to polymyositis (PM) or dermatomyositis (DM), we review the type of underlying ILD and assess the severity of respiratory impairment and the rate of progression.

Overview — Not every patient with ILD due to PM or DM requires treatment of the ILD as most patients follow a chronic, slowly-progressive course [1]. While formal guidelines are not available, we observe patients without treatment in the setting of the following characteristics: mild dyspnea on exertion, <10 percent involvement on high resolution computed tomography, forced vital capacity >75 percent of predicted, and/or diffusing capacity for carbon monoxide (DLCO) >65 percent of predicted [2]. However, for other patients with a greater degree of respiratory impairment and more pronounced abnormalities on imaging and pulmonary function tests, immunosuppressive therapy for ILD is generally indicated.

For patients with DM or PM who are receiving immunosuppressive therapy for their myositis, it is essential to exclude infection or drug-induced pulmonary toxicity as causes of lung disease. In addition, patients with DM and PM are at increased risk of malignancy, including cancers involving the lungs [3]. Thus, it is prudent to ascertain that the patient has undergone appropriate cancer screening. (See "Interstitial lung disease in dermatomyositis and polymyositis: Clinical manifestations and diagnosis", section on 'Differential diagnosis' and "Malignancy in dermatomyositis and polymyositis", section on 'Approach to screening'.)

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Literature review current through: Dec 2017. | This topic last updated: Jun 27, 2016.
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