Sarcoidosis-Associated Pulmonary Hypertension

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.

Sarcoidosis-associated pulmonary hypertension

“…sarcoidosis-associated pulmonary hypertension, SAPH) se uglavnom preuzimaju iz malih retrospektivnih studija. Procenjena prevalenca SAPH kreće se od 3% kod pacijenata upućenih u tercijarnu ustanovu do 79% kod pacijenata koji čekaju transplantaciju pluća [2]. Plućna hipertenzija je nezavisni prediktor mortaliteta kod bolesnika sa sarkoidozom [3].…”

“…Ima veliki značaj i u praćenju ovih bolesnika. Ehokardiografi ja može da preceni ili potceni pluć ni arterijski pritisak (PAP), posebno kod pacijenata sa intersticijumskom bolešć u pluć a [2].…”

“…Kateterizacija desnog srca je zlatni standard u dijagnostici plućne hipertenzije [2]. To je invazivna procedura kojom se plućna hipertenzija jasno definiše kada je srednji plućni arterijski pritisak u stanju mirovanja ≥20 mmHg [1].…”

Healthcare resource utilization and quality of life in patients with sarcoidosis‐associated pulmonary hypertension

“…1 , 3 , 4 , 5 Pulmonary hypertension (PH) is a well‐recognized and serious but poorly defined complication of sarcoidosis. 2 , 4 , 6 Estimates from prospective screening studies on the prevalence of PH in patients with sarcoidosis range from 3% to 6% when PH is confirmed by right heart catheterization (RHC) and from 6% to 21% for PH suspected by echocardiography. 2 , 7 , 8 , 9 , 10 , 11 , 12 …”

“…Non‐caseating granulomas develop in multiple tissues, commonly affecting the lungs and lymphatic system and potentially causing severe morbidity 1,3–5 . Pulmonary hypertension (PH) is a well‐recognized and serious but poorly defined complication of sarcoidosis 2,4,6 . Estimates from prospective screening studies on the prevalence of PH in patients with sarcoidosis range from 3% to 6% when PH is confirmed by right heart catheterization (RHC) and from 6% to 21% for PH suspected by echocardiography 2,7–12 …”