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Updated: Jul 2, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pulmonary hypertension in interstitial lung disease
1Advanced Lung Disease and Transplant Program, Inova Heart and Vascular Institute, Inova Fairfax Hospital, 3300 Gallows Road, Falls Church, VA 22042, USA. steven.nathan@inova.org
Abstract:
Pulmonary hypertension (PH) may complicate the course of many forms of advanced interstitial lung disease (ILD) and has been shown to portend a worse outcome. The aetiology of PH is likely multifactorial with variable contribution of factors amongst the different diseases. The most common such conditions include idiopathic pulmonary fibrosis, sarcoidosis, connective tissue disease-related ILD and pulmonary langerhans cell histiocytosis. Whether the course and impact of PH in these conditions can be modified by therapy requires further study.
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