{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T16:22:39Z","timestamp":1790266959120,"version":"4.1.0"},"reference-count":102,"publisher":"Georg Thieme Verlag KG","issue":"05","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Semin Respir Crit Care Med"],"published-print":{"date-parts":[[2020,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Pulmonary hypertension (PH) is a well-known complication of sarcoidosis, defined by a mean pulmonary artery pressure of \u226525\u2009mm 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.<\/jats:p>","DOI":"10.1055\/s-0040-1713615","type":"journal-article","created":{"date-parts":[[2020,8,10]],"date-time":"2020-08-10T22:35:42Z","timestamp":1597098942000},"page":"659-672","source":"Crossref","is-referenced-by-count":20,"title":["Sarcoidosis-Associated Pulmonary Hypertension"],"prefix":"10.1055","volume":"41","author":[{"given":"Marloes P.","family":"Huitema","sequence":"additional","affiliation":[{"name":"Department of Cardiology, St. Antonius Hospital, Nieuwegein, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harold","family":"Mathijssen","sequence":"additional","affiliation":[{"name":"Department of Cardiology, St. Antonius Hospital, Nieuwegein, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johannes J.","family":"Mager","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, St. Antonius Hospital, Nieuwegein, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Repke J.","family":"Snijder","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, St. Antonius Hospital, Nieuwegein, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan C.","family":"Grutters","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, St. Antonius Hospital, Nieuwegein, The Netherlands"},{"name":"Department of Pulmonology, University Medical Centre Utrecht, Utrecht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco C.","family":"Post","sequence":"additional","affiliation":[{"name":"Department of Cardiology, St. Antonius Hospital, Nieuwegein, The Netherlands"},{"name":"Department of Cardiology, University Medical Centre Utrecht, Utrecht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2020,8,10]]},"reference":[{"issue":"01","key":"ref1","doi-asserted-by":"crossref","first-page":"153","DOI":"10.7326\/0003-4819-31-1-153","article-title":"Boeck's sarcoid; a case of sarcoidosis complicated by pulmonary emphysema and cor pulmonale","volume":"31","author":"I Zimmerman","year":"1949","journal-title":"Ann Intern Med"},{"issue":"01","key":"ref2","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1093\/eurheartj\/ehv317","article-title":"2015 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"37","author":"N Gali\u00e8","year":"2016","journal-title":"Eur Heart J"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"D42","DOI":"10.1016\/j.jacc.2013.10.032","article-title":"Definitions and diagnosis of pulmonary hypertension","volume":"62","author":"M M Hoeper","year":"2013","journal-title":"J Am Coll Cardiol"},{"issue":"01","key":"ref4","doi-asserted-by":"crossref","first-page":"1801913","DOI":"10.1183\/13993003.01913-2018","article-title":"Haemodynamic definitions and updated clinical classification of pulmonary hypertension","volume":"53","author":"G Simonneau","year":"2019","journal-title":"Eur Respir J"},{"issue":"129","key":"ref5","doi-asserted-by":"crossref","first-page":"292","DOI":"10.1183\/09059180.00002713","article-title":"Pulmonary hypertension in chronic interstitial lung diseases","volume":"22","author":"A Caminati","year":"2013","journal-title":"Eur Respir Rev"},{"issue":"05","key":"ref6","doi-asserted-by":"crossref","first-page":"1246","DOI":"10.1378\/chest.129.5.1246","article-title":"Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis","volume":"129","author":"T Handa","year":"2006","journal-title":"Chest"},{"issue":"02","key":"ref7","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1183\/09031936.00175907","article-title":"Clinical predictors of pulmonary hypertension in sarcoidosis","volume":"32","author":"J M Bourbonnais","year":"2008","journal-title":"Eur Respir J"},{"issue":"02","key":"ref8","doi-asserted-by":"crossref","first-page":"86","DOI":"10.4103\/1817-1737.62471","article-title":"Sarcoidosis-associated pulmonary hypertension: clinical features and outcomes in Arab patients","volume":"5","author":"E H Alhamad","year":"2010","journal-title":"Ann Thorac Med"},{"issue":"04","key":"ref9","doi-asserted-by":"crossref","first-page":"1900897","DOI":"10.1183\/13993003.00897-2019","article-title":"Prevalence of pulmonary hypertension in pulmonary sarcoidosis: the first large European prospective study","volume":"54","author":"M P Huitema","year":"2019","journal-title":"Eur Respir J"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.clim.2018.12.012","article-title":"The long-term prognostic significance of sarcoidosis-associated pulmonary hypertension - 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