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Interstitial lung disease in scleroderma.


ABSTRACT: Systemic sclerosis is a heterogeneous disease of unknown etiology with limited effective therapies. It is characterized by autoimmunity, vasculopathy, and fibrosis and is clinically manifested by multiorgan involvement. Interstitial lung disease is a common complication of systemic sclerosis and is associated with significant morbidity and mortality. The diagnosis of interstitial lung disease hinges on careful clinical evaluation and pulmonary function tests and high-resolution computed tomography. Effective therapeutic options are still limited. Several experimental therapies are currently in early-phase clinical trials and show promise.

SUBMITTER: Schoenfeld SR 

PROVIDER: S-EPMC4385198 | biostudies-literature | 2015 May

REPOSITORIES: biostudies-literature