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Sarcoidosis-Associated Pulmonary Hypertension
Marloes P Huitema1, Harold Mathijssen1, Johannes J Mager2
1Department of Cardiology, St. Antonius Hospital, Nieuwegein, The Netherlands.
Seminars in Respiratory and Critical Care Medicine
|August 11, 2020
Summary
Sarcoidosis-associated pulmonary hypertension (SAPH) is a rare complication with variable prevalence. Diagnosis and treatment of SAPH are challenging, impacting patient morbidity and mortality.
Area of Science:
- Cardiology
- Pulmonology
- Rheumatology
Background:
- Pulmonary hypertension (PH) is a known complication of sarcoidosis.
- Sarcoidosis-associated PH (SAPH) data comes from small retrospective studies.
- SAPH prevalence varies widely, from 3% to 79% in specific patient groups.
Purpose of the Study:
- To review the current understanding of sarcoidosis-associated pulmonary hypertension.
- To discuss diagnostic challenges and potential mechanisms of SAPH.
- To outline current and potential treatment strategies for SAPH.
Main Methods:
- Review of existing literature on sarcoidosis-associated pulmonary hypertension.
- Analysis of reported prevalence, mechanisms, diagnostic approaches, and treatments.
- Synthesis of data from retrospective studies and clinical observations.
Main Results:
- Advanced parenchymal disease is the most common mechanism for SAPH.
- Other contributing factors include vascular disease, embolisms, and comorbidities.
- Diagnosis is challenging due to overlapping symptoms; echocardiography is key for screening.
Conclusions:
- SAPH significantly increases morbidity and mortality in sarcoidosis patients.
- Treatment requires a multidisciplinary approach in expert centers.
- Further research is needed due to scarce qualitative evidence on treatment efficacy.
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