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Updated: Sep 19, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Connective tissue disease-related interstitial lung disease and pulmonary hypertension
Caroline Motschwiller1, David Morales1, Roxana Sulica1
1NYU Langone Health, NYU Langone Grossman School of Medicine, Department of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, New York, NY, USA.
Abstract:
Pulmonary hypertension (PH) is an important cause of morbidity and mortality in connective tissue diseases (CTDs) and may develop either in isolation or in combination with interstitial lung disease (ILD). Based on the World Health Organization PH classification into five groups, patients with CTDs typically belong either to group 1 pulmonary arterial hypertension or to group 3 PH due to chronic lung disease and hypoxaemia (PH-ILD). Recent epidemiological studies have delineated the phenotypical complexity of CTD patients who present with both PH and ILD. This review explores the prevalence, diagnosis, pathophysiology, survival and management strategies for CTD-ILD-PH.
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