Journal
RHEUMATIC DISEASE CLINICS OF NORTH AMERICA
Volume 41, Issue 2, Pages 237-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.rdc.2014.12.005
Keywords
Systemic sclerosis; Interstitial lung disease; Fibrosis; Pathogenesis; Diagnosis; Treatment
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Funding
- NIAMS NIH HHS [T32 AR007258, K08 AR062592] Funding Source: Medline
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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.
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