Pulmonary hypertension associated with combined fibrosing mediastinitis and bronchial anthracofibrosis: A retrospective analysis in a single Chinese hospital

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.