Title:Interstitial Lung Disease in Systemic Sclerosis: Pathophysiology, Current and New Advances in Therapy
VOLUME: 11 ISSUE: 4
Author(s):Snigdha Jain, Anupama Shahane and Chris T. Derk
Affiliation:Division of Rheumatology, University of Pennsylvania, 8th Floor-Penn Tower, One Convention Blvd., Philadelphia, PA 19104, USA.
Keywords:Interstitial lung disease, lung, therapy, pathophysiology, scleroderma, systemic sclerosis, visceral organs, cyclophosphamide, pulmonary fibrosis, Diffuse cutaneous SSc, limited cutaneous SSc
Abstract:Systemic sclerosis is an autoimmune connective tissue disorder characterized by fibrosis of the skin and
visceral organs. Interstitial lung disease (ILD) is a major complication of this disease and along with pulmonary arterial
hypertension is the leading cause of mortality in scleroderma patients. The pathogenesis of pulmonary fibrosis is
characterized by epithelial cell injury, activation of the coagulation pathway and inflammation, which create a
profibrogenic environment in the lung in the setting of autoimmunity. The current standard of treatment for ILD in
systemic sclerosis is cyclophosphamide. In view of the modest benefits in pulmonary function seen with
cyclophosphamide in two recent trials and its significant toxicity, the search for alternative treatments is ongoing. With
the advances in our understanding of the pathogenic mechanisms of pulmonary fibrosis, many promising therapeutic
agents have come into view, but their efficacy needs to be evaluated before they can be recommended clinically. This
review discusses the pathogenesis of pulmonary fibrosis with a focus on the potential target pathways, the current
treatment options and recent advances in the treatment of ILD in systemic sclerosis.
