Determining progression of scleroderma-related interstitial lung disease

Elizabeth R Volkmann; Donald P Tashkin; Myung Sim; Grace Hyun Kim; Jonathan Goldin; Philip J Clements

doi:10.1177/2397198318816915

Determining progression of scleroderma-related interstitial lung disease

J Scleroderma Relat Disord. 2019 Feb;4(1):62-70. doi: 10.1177/2397198318816915. Epub 2018 Dec 17.

Authors

Elizabeth R Volkmann¹, Donald P Tashkin¹, Myung Sim¹, Grace Hyun Kim^{2

3}, Jonathan Goldin², Philip J Clements¹

Affiliations

¹ Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA.
² Department of Radiology, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA.
³ Department of Biostatistics, Fielding School of Public Health, University of California, Los Angeles, Los Angeles, CA, USA.

Abstract

Interstitial lung disease occurs in the majority of patients with systemic sclerosis. Although interstitial lung disease is the number one cause of death in systemic sclerosis, interstitial lung disease progression rates vary considerably among patients with systemic sclerosis. Some patients with systemic sclerosis-associated interstitial lung disease have sub-clinical disease and may not derive benefit from immunosuppression, while others have a more aggressive interstitial lung disease phenotype. Reliable predictors of interstitial lung disease progression are lacking. The present review describes our current approach to monitoring systemic sclerosis-associated interstitial lung disease progression in clinical practice. To illustrate the marked heterogeneity that exists in interstitial lung disease progression rates in systemic sclerosis, this review presents the individual disease course of five unique patients with systemic sclerosis-associated interstitial lung disease who participated in the Scleroderma Lung Study II. These cases illustrate that treatment response rates vary in systemic sclerosis-associated interstitial lung disease and more research is needed to determine how to predict treatment response in systemic sclerosis-associated interstitial lung disease and to develop personalized treatment approaches for patients with this devastating disease.

Keywords: Systemic sclerosis; interstitial lung disease; survival; treatment.