Related Experiment Video
Updated: Aug 19, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pulmonary Arterial Hypertension in Connective Tissue Diseases Beyond Systemic Sclerosis
Christopher Lewis1, Ryan Sanderson2, Nektarios Vasilottos3
1Department of Internal Medicine, Division of Cardiovascular Medicine, Henry Ford Hospital, Detroit, MI, USA.
Abstract:
Pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD) (CTD-PAH) is a devastating condition that may progress rapidly to cause right ventricular dysfunction, resulting in significant morbidity and mortality. The pathobiology, epidemiology, natural history, early diagnosis, and treatment response of PAH associated with scleroderma (SSc-PAH) have been the subjects of intense research efforts over the previous decade. The success of these efforts has resulted in increased awareness and earlier detection of SSc-PAH. Practitioners are less aware of the risk of PAH associated with other CTDs; the aim of this article is to discuss the broader scope of CTD-PAH.
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