Orphanet journal of rare diseases · 2025
Long-term outcomes and prognostic predictors in patients with fibrosing mediastinitis associated pulmonary hypertension: a multicenter cohort study.
СтатьяМногоцентровое исследованиеПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Zhang Hanwen, Yang Beilan, Liu Bingyang, Luo Qin, Tang Yinjiang, Wang Ting, Jiang Gaxue, Xi Qunying, Wang Yinli, Ruan Hongyun, Nan Jinglong, Yang Yicheng, Zeng Qixian, Yan Wenjie, Liang Yanru, Zhao Zhihui, Yang Tao, Chen Yu, Wu Yan, Tao Xincao, Zhou Xiaoming, Liu Zhihong, You Lingyan, Xiong Changming
Аннотация
BACKGROUND: Fibrosing mediastinitis (FM) is a rare condition that may be complicated by pulmonary hypertension (PH). 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).
METHODS: 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.
RESULTS: 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 11-55], 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%.
CONCLUSION: 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.
METHODS: 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.
RESULTS: 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 11-55], 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%.
CONCLUSION: 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.