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Pulmonary hypertension associated with combined fibrosing mediastinitis and bronchial anthracofibrosis: A retrospective analysis in a single Chinese hospital
Authors:Yan Xu  Wenbing Xu  Yongjian Liu  Xinlun Tian  Kaifeng Xu  Juhong Shi  Mengzhao Wang  Baiqiang Cai  Min Peng
Affiliation:Department of Respiratory Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Abstract:

Introduction

Both fibrosing mediastinitis (FM) and bronchial anthracofibrosis (BAF) are unique diseases. The combined appearance of FM and BAF is extremely rare.

Objectives

The aim of this study was to investigate the clinical features of patients with coexisting FM and BAF.

Method

Between January 2003 and December 2015, a total of eight patients were diagnosed at the Peking Union Medical College Hospital as having combined FM and BAF. The clinical presentations, radiographic features and bronchoscopic findings of the eight patients were reviewed.

Results

The patients were five women and three men with a median age of 64 years (range 56‐86 years). Symptoms included dyspnea (eight patients), cough (seven patients), chest pain (two patients), hemoptysis (two patients) and so on. Chest CT of all eight patients showed mediastinal soft‐tissue lesions, with multiple narrowed or obliterated lobar or segmental bronchi and arteries. Bronchoscopy showed that all of the patients had multiple stenoses of lobar or segmental bronchi with anthracotic pigmentation on the mucosa. Echocardiography showed that all of the patients had elevated pulmonary arterial systolic pressure (median 81 mm Hg, range 51‐107 mm Hg). Each of the eight patients had a history of exposure to, or infection with, tuberculosis, although there was no evidence of active disease. All of the eight patients had long‐term exposure to indoor coal or biomass fuel smoke.

Conclusions

FM can coexist with BAF, characterized by prominent pulmonary hypertension. The possible etiological factors are tuberculosis and coal or biomass fuel exposure.
Keywords:biomass fuel  bronchial anthracofibrosis  fibrosing mediastinitis  pulmonary hypertension  tuberculosis
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