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This multicenter study aimed to investigate the clinical features, long-term survival outcomes, and prognostic factors in patients with fibrosing mediastinitis associated pulmonary hypertension (FM-PH).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A total of 85 FM-PH patients were enrolled across seven centers in China between January 2007 and July 2024. Patients were classified into two groups based on the occurence of clinical worsening (CW): FM-PH with CW and FM-PH without CW. Clinical worsening was defined as a composite of all-cause mortality, rehospitalization for heart failure, or deterioration in World Health Organization functional class (WHO-FC) compared with baseline.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Of the 85 FM-PH patients, 37 were classified as FM-PH without CW and 48 as FM-PH with CW. The FM-PH with CW patients had significantly higher levels of systemic inflammation, elevated N-terminal pro-brain natriuretic peptide (NT-proBNP), higher mean pulmonary artery pressure (mPAP), and worse right heart function compared to the FM-PH without CW patients. Multivariate Cox regression analysis showed that high-sensitivity C-reactive protein (hs-CRP), mPAP, and peripheral edema were independently associated with clinical worsening in FM-PH patients. Over a median follow-up of 27 months [IQR 11\u201355], 48 patients experienced clinical worsening events, including five deaths. The 1-, 3-, and 5-year overall survival rates were 94.5%, 86.3%, and 84.6%, respectively. However, the 5-year clinical worsening-free rate was only 26.6%.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Although the 1-, 3-, and 5-year overall survival rates were relatively favorable, the 5-year clinical worsening-free rate was not satisfying. Elevated hs-CRP, mPAP and the presence of peripheral edema were independently associated with worse clinical outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s13023-025-04066-8","type":"journal-article","created":{"date-parts":[[2025,11,12]],"date-time":"2025-11-12T14:28:39Z","timestamp":1762957719000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Long-term outcomes and prognostic predictors in patients with fibrosing mediastinitis associated pulmonary hypertension: a multicenter cohort study"],"prefix":"10.1186","volume":"20","author":[{"given":"Hanwen","family":"Zhang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beilan","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bingyang","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qin","family":"Luo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yinjiang","family":"Tang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ting","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gaxue","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qunying","family":"Xi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yinli","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hongyun","family":"Ruan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jinglong","family":"Nan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yicheng","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qixian","family":"Zeng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wenjie","family":"Yan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yanru","family":"Liang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhihui","family":"Zhao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tao","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yan","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xincao","family":"Tao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoming","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhihong","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lingyan","family":"You","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Changming","family":"Xiong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,11,12]]},"reference":[{"issue":"3","key":"4066_CR1","doi-asserted-by":"publisher","first-page":"651","DOI":"10.1148\/rg.2019180143","volume":"39","author":"SH Garrana","year":"2019","unstructured":"Garrana SH, Buckley JR, Rosado-de-Christenson ML, Mart\u00ednez-Jim\u00e9nez S, Mu\u00f1oz P, Borsa JJ. 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