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Updated: May 9, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Sarcoidosis-associated pulmonary hypertension
Francis C Cordova1, Gilbert D'Alonzo
1Division of Pulmonary and Critical Care Medicine, Medical Director, Lung Transplant Program, Temple University School of Medicine, Philadelphia, PA 19140, USA. cordovf@tuhs.temple.edu
Current Opinion in Pulmonary Medicine
|August 6, 2013
Summary
Sarcoid-associated pulmonary hypertension (SAPH) is common, even in early disease stages. Early screening and treatment with vasodilators and anti-inflammatory agents can improve quality of life for SAPH patients.
Area of Science:
- Pulmonary Medicine
- Cardiology
- Rheumatology
Background:
- Pulmonary hypertension (PH) is a severe complication of sarcoidosis.
- Sarcoid-associated pulmonary hypertension (SAPH) can affect patients with varying disease severity.
Purpose of the Study:
- To review the clinical characteristics, diagnostic challenges, and therapeutic strategies for SAPH.
- To highlight the importance of early screening and management of SAPH.
Main Methods:
- Review of clinical characteristics, risk factors, and diagnostic tools for SAPH.
- Analysis of treatment outcomes with pulmonary vasodilators and anti-inflammatory agents.
- Evaluation of prognostic indicators such as forced vital capacity.
Main Results:
- SAPH is associated with restrictive lung physiology, hypoxemia, advanced chest X-ray stage, and low diffusing capacity.
- Echocardiography is a useful screening tool, but right heart catheterization is definitive for diagnosis.
- Pulmonary vasodilators may improve hemodynamics but not always exercise capacity; different SAPH phenotypes exist.
Conclusions:
- Persistent dyspnea in sarcoidosis patients warrants screening for PH.
- SAPH carries a poor prognosis, necessitating consideration for lung transplantation.
- Combined anti-inflammatory and vasodilator therapy can improve symptoms, function, and quality of life in some SAPH patients.
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