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Updated: Jan 14, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Genetic Familial Interstitial Lung Disease
Rafael J Fernandez1, Jonathan A Kropski2
1Division of Allergy, Pulmonary and Critical Care Medicine, Department of Medicine, Vanderbilt University Medical Center, 1161 21st Avenue S, T1218 MCN, Nashville, TN 37232, USA. Electronic address: https://twitter.com/rjfernandeziii.
Abstract:
Interstitial lung diseases (ILDs) are a heterogenous group of disorders leading to progressive loss of lung function. A subset of ILD cases can be linked to specific single-gene causes. The available evidence suggests that known genetic etiologies should influence pharmacotherapy decisions for ILD patients, particularly when immunosuppression is considered. There is emerging consensus supporting screening of unaffected relatives of familial ILD patients to enhance early disease detection, while future studies exploring primary and secondary prevention of ILD in high-risk individuals offer hope of preventing the life-limited complications of these disorders.
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