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Review
. 2025 Feb 14;61(2):342.
doi: 10.3390/medicina61020342.

Sarcoidosis-Associated Pulmonary Hypertension

Yoshitaka Morimatsu  1 , Nobuhiro Tahara  2 , Masaki Okamoto  3   4 , Munehisa Bekki  2 , Atsuko Tahara  2 , Yoshiko Eto  2 , Tadahiro Kugai  2 , Yuki Koga  2 , Shoko Maeda-Ogata  2 , Akihiro Honda  2 , Sachiyo Igata  2 , Yoshiaki Zaizen  4 , Shuichi Tanoue  5 , Tomoaki Hoshino  4 , Tatsuya Ishitake  1 , Yoshihiro Fukumoto  2
Affiliations
Review

Sarcoidosis-Associated Pulmonary Hypertension

Yoshitaka Morimatsu et al. Medicina (Kaunas). .

Abstract

Sarcoidosis is a granulomatous disorder of unknown etiology characterized by multisystem non-caseating granulomas. Pulmonary hypertension (PH) is a well-known complication of sarcoidosis and is associated with increased morbidity and mortality. The actual epidemiology of sarcoidosis-associated PH (SAPH) remains unknown, and its pathogenesis has not been fully elucidated. SAPH is classified under the miscellaneous category (group 5 of the PH classification). The clinical presentation of SAPH is variable and not always proportional to the severity of sarcoidosis. Appropriate management for SAPH by an experienced physician is important; however, no treatment algorithm for SAPH has been established. Lung transplantation should be considered in refractory cases. Pulmonary arterial hypertension-specific vasodilators targeting the endothelin pathway, nitric oxide pathway, and prostacyclin pathway have improved the clinical functions and hemodynamics in some patients with SAPH.

Keywords: epidemiology; pathogenesis; sarcoidosis; sarcoidosis-associated pulmonary hypertension; treatment.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Screening algorithm of SAPH. CT, computed tomography; SAPH, sarcoidosis-associated pulmonary hypertension; PA, pulmonary artery; AA, ascending aorta; PASP, pulmonary artery systolic pressure; PAP, pulmonary arterial pressure; RV, right ventricle.
Figure 2
Figure 2
Transthoracic echocardiographic images of a patient with SAPH. Transthoracic echocardiography demonstrates a septal thinning (arrow) and regurgitant tricuspid jet, suggesting pulmonary hypertension in a patient with cardiac sarcoidosis. SAPH, sarcoidosis-associated pulmonary hypertension; RA, right atrium; RV, right ventricle; LA, left atrium; LV, left ventricle.
Figure 3
Figure 3
Chest X-ray and FDG-PET images according to the Scadding stage. 18F-Fluorodeoxyglucose-positron emission tomography (FDG-PET) showing FDG uptake in inflammatory lesions of pulmonary sarcoidosis.
Figure 4
Figure 4
Representative sarcoidosis cases with FDG uptake in the heart. 18F-Fluorodeoxyglucose-positron emission tomography (FDG-PET) showing FDG uptake in the left ventricle (A). FDG uptake is shown in the right and left ventricle (B). FDG uptake lesion were pointed by red arrow. CT, computed tomography; MIP, maximum intensity projection; RA, right atrium; RV, right ventricle; LA, left atrium; LV, left ventricle.
See this image and copyright information in PMC

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