Abstract
Pulmonary hypertension (PH) is a well-known complication of sarcoidosis, defined by
a mean pulmonary artery pressure of ≥25 mm Hg. Since both PH and sarcoidosis are rare
diseases, data on sarcoidosis-associated PH (SAPH) is retrieved mostly from small
retrospective studies. Estimated prevalence of SAPH ranges from 3% in patients referred
to a tertiary center up to 79% in patients awaiting lung transplant. Most patients
with SAPH show advanced parenchymal disease as the underlying mechanism. However,
some patients have disproportional elevated pulmonary artery pressure, and PH can
occur in sarcoidosis patients without parenchymal disease. Other mechanisms such as
vascular disease, pulmonary embolisms, postcapillary PH, extrinsic compression, and
other sarcoidosis-related comorbidities might contribute to SAPH. The diagnosis of
PH in sarcoidosis is challenging since symptoms and signs overlap. Suspicion can be
raised based on symptoms or tests, such as pulmonary function tests, laboratory findings,
electrocardiography, or chest CT. PH screening mainly relies on transthoracic echocardiography.
Right heart catheterization should be considered on a case-by-case basis in patients
with clinical suspicion of PH, taking into account clinical consequences. Treatment
options are considered on patient level in a PH expert center, and might include oxygen
therapy, immunosuppressive, or PH-specific therapy. However, qualitative evidence
is scarce. Furthermore, in a subset of patients, interventional therapy or eventually
lung transplant can be considered. SAPH is associated with high morbidity. Mortality
is higher in sarcoidosis patients with PH compared with those without PH, and increases
in patients with more advanced stages of sarcoidosis and/or PH.
Keywords
pulmonary hypertension - sarcoidosis - sarcoidosis-associated pulmonary hypertension
