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Rheum Dis Clin North Am. 2015 May;41(2):237-48. doi: 10.1016/j.rdc.2014.12.005. Epub 2015 Feb 26.

Interstitial lung disease in scleroderma.

Author information

1
Division of Rheumatology, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA.
2
Division of Rheumatology, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA. Electronic address: fcastelino@mgh.harvard.edu.

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.

KEYWORDS:

Diagnosis; Fibrosis; Interstitial lung disease; Pathogenesis; Systemic sclerosis; Treatment

PMID:
25836640
PMCID:
PMC4385198
DOI:
10.1016/j.rdc.2014.12.005
[Indexed for MEDLINE]
Free PMC Article

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