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Immunosuppressive Treatment Regimens in Connective Tissue Disease–associated Interstitial Lung Disease

Hirsh, Brian, MD; Lande, Leah, MD

doi: 10.1097/CPM.0000000000000282
Interstitial, Inflammatory and Occupational Lung Disease

The management of interstitial lung disease due to connective tissue disease presents a challenge for clinicians, as this is a heterogeneous group of diseases, and there are no clear guidelines to follow, with only sparse randomized controlled trials available to guide therapy. Multiple different medications have been used for the treatment of interstitial lung disease due to connective tissue disease, with mostly retrospective studies or small case series documenting their efficacy. Ultimately, the choice of medication, the dosage, and the duration of therapy should be individualized for each patient after weighing the risks of adverse drug effects versus the risk of disease progression. In this review, different immunosuppressive regimens are profiled, focusing on the available data with regard to the efficacy of each medication, potential adverse effects, and recommended drug monitoring.

Division of Pulmonary and Critical Care Medicine, Lankenau Medical Center, Wynnewood, PA

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

Address correspondence to: Brian Hirsh, MD, 100 E. Lancaster Ave, Suite 230, Wynnewood, PA 19096. E-mail:

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