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Review: Interstitial Lung Disease Associated With Systemic Sclerosis and Idiopathic Pulmonary Fibrosis: How Similar and Distinct?
Author(s) -
Herzog Erica L.,
Mathur Aditi,
Tager Andrew M.,
FeghaliBostwick Carol,
Schneider Frank,
Varga John
Publication year - 2014
Publication title -
arthritis and rheumatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.106
H-Index - 314
eISSN - 2326-5205
pISSN - 2326-5191
DOI - 10.1002/art.38702
Subject(s) - interstitial lung disease , pulmonary fibrosis , medicine , idiopathic pulmonary fibrosis , scleroderma (fungus) , pathology , lung , pulmonary disease , disease , lung disease , fibrosis , inoculation
Fibrosis of the lung, a common complication of systemic sclerosis (SSc) and the hallmark of idiopathic pulmonary fibrosis (IPF), is associated with substantial mortality and has no approved therapy. Despite some degree of overlap in their clinical features and pathogenesis, SSc-associated interstitial lung disease (ILD) and IPF have differences, with significant implications for diagnosis, evaluation, and management. To shed light on these issues, this review compares and contrasts salient features of these 2 entities, focusing on clinical manifestations, lung imaging, and pathology, along with current concepts of pathogenesis, including animal models, translational studies, genetic factors, and predictive biomarkers. We conclude by posing questions that might unveil new areas of investigation and inform novel and targeted approaches to therapy.