Interstitial lung disease in dermatomyositis and polymyositis: Treatment
- Paul F Dellaripa, MD
Paul F Dellaripa, MD
- Associate Professor of Medicine
- Harvard Medical School
- Marc L Miller, MD
Marc L Miller, MD
- Clinical Assistant Professor of Medicine
- Tufts University School of Medicine
- Section Editors
- Ira N Targoff, MD
Ira N Targoff, MD
- Section Editor — Muscle Disease
- Professor of Medicine, Section of Rheumatology
- University of Oklahoma Health Sciences Center
- Kevin R Flaherty, MD, MS
Kevin R Flaherty, MD, MS
- Section Editor — Interstitial Lung Disease
- Associate Professor of Medicine
- University of Michigan Health System
- Deputy Editors
- Helen Hollingsworth, MD
Helen Hollingsworth, MD
- Deputy Editor — Pulmonary, Critical Care, and Sleep Medicine
- Associate Professor of Medicine
- Boston University School of Medicine
- Monica Ramirez Curtis, MD, MPH
Monica Ramirez Curtis, MD, MPH
- Deputy Editor — Rheumatology
- Instructor of Medicine
- Harvard Medical School
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".)
APPROACH TO THERAPY
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 . 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 . 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 . 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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- APPROACH TO THERAPY
- Nonspecific interstitial pneumonia
- Organizing pneumonia
- Other types of ILD
- INITIAL GLUCOCORTICOID THERAPY FOR NSIP
- ADDING A SECOND AGENT
- Choice of agent
- - Azathioprine
- - Mycophenolate mofetil
- - Calcineurin inhibitors
- - Methotrexate
- REFRACTORY DISEASE
- Combination therapy
- Intravenous immune globulin
- LUNG TRANSPLANTATION
- ASSESSING THE RESPONSE AND DURATION OF THERAPY
- Spontaneous pneumomediastinum
- Prevention of treatment related complications
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS