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.
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Herzog et al. (2014) studied this question.