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Updated: Jul 3, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pulmonary hypertension in interstitial lung disease
David Polomis1, James R Runo, Keith C Meyer
1Department of Medicine, University of Wisconsin, School of Medicine and Public Health, Madison, Wisconsin 53792-9988, USA.
Pulmonary hypertension associated with interstitial lung disease (PH-ILD) is increasingly recognized. New treatments offer hope, but long-term outcomes and optimal timing remain uncertain.
Area of Science:
- Pulmonary Medicine
- Cardiology
- Respiratory Diseases
Background:
- Pulmonary hypertension associated with interstitial lung disease (PH-ILD) is a significant clinical challenge.
- Understanding the prevalence, pathophysiology, and natural history of PH-ILD is crucial for effective management.
Purpose of the Study:
- To review recent advancements in the understanding of PH-ILD.
- To explore current and emerging treatment strategies for PH-ILD.
Main Methods:
- Literature review of recent studies on PH-ILD.
- Analysis of data on pathophysiology, diagnosis, and treatment outcomes.
Main Results:
- Significant progress in understanding PH-ILD etiology and pathophysiology.
- New therapeutic agents, initially for idiopathic pulmonary arterial hypertension, are being applied to PH-ILD.
- Short-term benefits of advanced therapies are clearer, but long-term data and combination therapy efficacy are lacking.
- Lung transplantation outcomes for PH-ILD have been further defined.
Conclusions:
- PH-ILD is frequently recognized and linked to considerable morbidity and mortality.
- Pharmacotherapies and lung transplantation expand treatment options for PH-ILD.
- Uncertainty remains regarding the optimal timing of advanced therapies and their long-term benefits and toxicities.
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