{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T02:20:30Z","timestamp":1787019630632,"version":"build-2736575974"},"reference-count":41,"publisher":"European Respiratory Society (ERS)","issue":"170","license":[{"start":{"date-parts":[[2023,12,20]],"date-time":"2023-12-20T00:00:00Z","timestamp":1703030400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir Rev"],"accepted":{"date-parts":[[2023,8,16]]},"published-print":{"date-parts":[[2023,12,31]]},"abstract":"<jats:p>Ever since the second world symposium on pulmonary hypertension (PH) held in Evian, France, in 1998, PH has been classified into five major clinical groups. Group 5 PH includes a variety of distinct conditions with unclear and\/or multifactorial underlying pathologies. Management of these patients is challenging as the number of patients within these groups is often small, not all individuals with certain underlying conditions are affected by PH and patients exhibit distinct symptoms due to different underlying diseases. Studies and clinical trials in these groups are largely lacking and mostly restricted to case series and registry reports. Nonetheless, the worldwide burden of group 5 PH is estimated to be significant in terms of the prevalence of some associated diseases. Group 5 PH encompasses six subgroups, including haematological disorders (inherited and acquired chronic haemolytic anaemia and chronic myeloproliferative disorders), systemic disorders (sarcoidosis, pulmonary Langerhans's cell histiocytosis and neurofibromatosis type 1), metabolic disorders (glycogen storage diseases and Gaucher disease), chronic renal failure with or without haemodialysis, pulmonary tumour thrombotic microangiopathy and fibrosing mediastinitis.<\/jats:p>","DOI":"10.1183\/16000617.0119-2023","type":"journal-article","created":{"date-parts":[[2023,12,20]],"date-time":"2023-12-20T04:40:18Z","timestamp":1703047218000},"page":"230119","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":7,"title":["Clinical\u2013radiological\u2013pathological correlation in pulmonary hypertension with unclear and\/or multifactorial mechanisms"],"prefix":"10.1183","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4485-1758","authenticated-orcid":false,"given":"Mona","family":"Lichtblau","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Mayer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8079-205X","authenticated-orcid":false,"given":"Deepa","family":"Gopalan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2499-6829","authenticated-orcid":false,"given":"Peter","family":"Dorfm\u00fcller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5250-5022","authenticated-orcid":false,"given":"Silvia","family":"Ulrich","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2023,12,20]]},"reference":[{"key":"2024102404162522000_32.170.230119.1","unstructured":"Rich S . Primary pulmonary hypertension. Executive summary from the World Symposium \u2013 Primary Pulmonary Hypertension 1998. Date last accessed: 13 September 2023. Date last updated: 16 September 2019. www.wsphassociation.org\/wp-content\/uploads\/2019\/04\/Primary-Pulmonary-Hypertension-Evian-1998.pdf"},{"key":"2024102404162522000_32.170.230119.2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2004.02.037"},{"key":"2024102404162522000_32.170.230119.3","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2013.10.029"},{"key":"2024102404162522000_32.170.230119.4","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":"Simonneau","year":"2018","journal-title":"Eur Respir J"},{"key":"2024102404162522000_32.170.230119.5","doi-asserted-by":"crossref","first-page":"3618","DOI":"10.1093\/eurheartj\/ehac237","article-title":"2022 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"43","author":"Humbert","year":"2022","journal-title":"Eur Heart J"},{"key":"2024102404162522000_32.170.230119.6","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehab368"},{"key":"2024102404162522000_32.170.230119.7","unstructured":"European Respiratory Society . Pulmonary hypertension: clinical\u2013radiological\u2013pathological case series. Date last accessed: 13 September 2023. Date last updated: 13 March 2023. https:\/\/www.ersnet.org\/events\/pulmonary-hypertension-clinical-radiological-pathological-case-series\/"},{"key":"2024102404162522000_32.170.230119.8","doi-asserted-by":"publisher","DOI":"10.1183\/16000617.0165-2021"},{"key":"2024102404162522000_32.170.230119.9","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201511-760OC"},{"key":"2024102404162522000_32.170.230119.10","doi-asserted-by":"publisher","DOI":"10.1378\/chest.09-2002"},{"key":"2024102404162522000_32.170.230119.11","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.04079-2020"},{"key":"2024102404162522000_32.170.230119.12","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01747-2019"},{"key":"2024102404162522000_32.170.230119.13","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00465-2017"},{"key":"2024102404162522000_32.170.230119.14","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2017.07.008"},{"key":"2024102404162522000_32.170.230119.15","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2005.042838"},{"key":"2024102404162522000_32.170.230119.16","doi-asserted-by":"publisher","DOI":"10.1378\/chest.128.3.1483"},{"key":"2024102404162522000_32.170.230119.17","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1016\/j.rmed.2018.04.015","article-title":"Clinical features of sarcoidosis associated pulmonary hypertension: results of a multi-national registry","volume":"139","author":"Baughman","year":"2018","journal-title":"Respir Med"},{"key":"2024102404162522000_32.170.230119.18","first-page":"74","article-title":"Safety of macitentan in sarcoidosis-associated pulmonary hypertension: a case-series","volume":"37","author":"Mathijssen","year":"2020","journal-title":"Sarcoidosis Vasc Diffuse Lung Dis"},{"key":"2024102404162522000_32.170.230119.19","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1159\/000519101","article-title":"Sickle cell disease-induced pulmonary hypertension: a review of pathophysiology, management, and current literature","volume":"9","author":"Sheikh","year":"2021","journal-title":"Pulse"},{"key":"2024102404162522000_32.170.230119.20","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-08-618561"},{"key":"2024102404162522000_32.170.230119.21","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00585-2019"},{"key":"2024102404162522000_32.170.230119.22","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00134410"},{"key":"2024102404162522000_32.170.230119.23","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1005565"},{"key":"2024102404162522000_32.170.230119.24","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-09-618587"},{"key":"2024102404162522000_32.170.230119.25","doi-asserted-by":"crossref","unstructured":"Aeberhard J , Lichtblau M , Arenja N , et al . An unexpected cause of pulmonary hypertension in a young woman \u2013 a case report. Eur Heart J Case Rep 2023; 7: ytad474. doi:10.1093\/ehjcr\/ytad474","DOI":"10.1093\/ehjcr\/ytad474"},{"key":"2024102404162522000_32.170.230119.26","doi-asserted-by":"crossref","first-page":"23247096221086453","DOI":"10.1177\/23247096221086453","article-title":"Pulmonary tumor embolism and pulmonary tumor thrombotic microangiopathy causing rapidly progressive respiratory failure: a case series","volume":"10","author":"Rajdev","year":"2022","journal-title":"J Investig Med High Impact Case Rep"},{"key":"2024102404162522000_32.170.230119.27","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(197510)36:4<1473::AID-CNCR2820360440>3.0.CO;2-D"},{"key":"2024102404162522000_32.170.230119.28","doi-asserted-by":"publisher","DOI":"10.1016\/1054-8807(92)90038-P"},{"key":"2024102404162522000_32.170.230119.29","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(68)90044-2"},{"key":"2024102404162522000_32.170.230119.30","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1186\/s12876-021-02009-8","article-title":"Pulmonary tumor thrombotic microangiopathy in occult early gastric cancer that was undetectable on upper endoscopy: a case report and review of similar cases","volume":"21","author":"Kawakami","year":"2021","journal-title":"BMC Gastroenterol"},{"key":"2024102404162522000_32.170.230119.31","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.52.9472"},{"key":"2024102404162522000_32.170.230119.32","doi-asserted-by":"publisher","DOI":"10.1536\/ihj.14-220"},{"key":"2024102404162522000_32.170.230119.33","doi-asserted-by":"crossref","first-page":"651","DOI":"10.2169\/internalmedicine.55.5758","article-title":"Pulmonary tumor thrombotic microangiopathy caused by urothelial carcinoma expressing vascular endothelial growth factor, platelet-derived growth factor, and osteopontin","volume":"55","author":"Wakabayashi","year":"2016","journal-title":"Intern Med"},{"key":"2024102404162522000_32.170.230119.34","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00050.2012"},{"key":"2024102404162522000_32.170.230119.35","doi-asserted-by":"crossref","first-page":"1621","DOI":"10.2169\/internalmedicine.3630-19","article-title":"A long-term survival case of pulmonary tumor thrombotic microangiopathy due to gastric cancer confirmed by the early diagnosis based on a transbronchial lung biopsy","volume":"59","author":"Imakura","year":"2020","journal-title":"Intern Med"},{"key":"2024102404162522000_32.170.230119.36","doi-asserted-by":"crossref","first-page":"1045","DOI":"10.1016\/j.rmr.2017.02.008","article-title":"Pulmonary tumor thrombotic microangiopathy","volume":"34","author":"Merad","year":"2017","journal-title":"Rev Mal Respir"},{"key":"2024102404162522000_32.170.230119.37","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1097\/MD.0000000000000219","article-title":"Pulmonary tumor thrombotic microangiopathy: report of 3 cases and review of the literature","volume":"93","author":"Gainza","year":"2014","journal-title":"Medicine"},{"key":"2024102404162522000_32.170.230119.38","doi-asserted-by":"publisher","DOI":"10.1016\/j.clbc.2014.10.008"},{"key":"2024102404162522000_32.170.230119.39","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.52.0718"},{"key":"2024102404162522000_32.170.230119.40","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.53.2379"},{"key":"2024102404162522000_32.170.230119.41","doi-asserted-by":"crossref","first-page":"e29412","DOI":"10.1097\/MD.0000000000029412","article-title":"Case report: apatinib plus selexipag as a novel therapy for pulmonary tumor thrombotic microangiopathy accompanied by pulmonary hypertension associated with gastric carcinoma","volume":"101","author":"Ma","year":"2022","journal-title":"Medicine"}],"container-title":["European Respiratory Review"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1183\/16000617.0119-2023","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,2,24]],"date-time":"2025-02-24T00:06:38Z","timestamp":1740355598000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.ersnet.org\/lookup\/doi\/10.1183\/16000617.0119-2023"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,12,20]]},"references-count":41,"journal-issue":{"issue":"170","published-online":{"date-parts":[[2023,12,20]]},"published-print":{"date-parts":[[2023,12,31]]}},"alternative-id":["10.1183\/16000617.0119-2023"],"URL":"https:\/\/doi.org\/10.1183\/16000617.0119-2023","relation":{},"ISSN":["0905-9180","1600-0617"],"issn-type":[{"value":"0905-9180","type":"print"},{"value":"1600-0617","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,12,20]]}}}