Related Experiment Video
Updated: May 26, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
An update on the evaluation and management of pulmonary hypertension in scleroderma
John G Coghlan1, Benjamin Schreiber, Benjamin Schrieber
1Department of Cardiology, Royal Free Hospital, London NW3 2QG, England, UK. gerry.coghlan@nhs.net
Abstract:
Pulmonary arterial hypertension associated with scleroderma (SScPAH) is a debilitating, highly lethal condition that responds to an array of therapies. Quality of life and prognosis are substantially improved by treatment, and early diagnosis and treatment are associated with improved outcomes. There are serious limitations to current screening programs. Many more questions need to be addressed. Why is PAH so common in SSc? Why is the tolerance of pulmonary hypertension so poor in scleroderma? What are the best measures of response to therapy in SSc patients with PAH? Should we use different parameters in prognostic scores in SScPAH? Why is postcapillary pulmonary hypertension so common in SSc? How do we reliably differentiate lung disease-associated pulmonary hypertension from PAH? The aim of this review is to summarize the main areas of progress over the past decade and to look to the challenges for the next decade.
Related Concept Videos
Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
Rheumatic Heart Disease IV: Nursing Management
Mitral Stenosis IV: Nursing Management
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Rheumatic Heart Disease III: Medical Management
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...

