Clinical features of primary tracheobronchial pulmonary amyloidosis

Abstract

Objective: To analyze the clinical features of primary tracheobronchial pulmonary amyloidosis (PTBA). Methods: The records of 11 patients with PTBA diagnosed by pathology from January 2002 to June 2018 in Xiangya Hospital of Central South University were retrospectively reviewed, including clinical manifestations, laboratory and imaging examination, bronchoscopic manifestations and therapies. Meanwhile, PTBA was staged based on the severity and extent of lesion under bronchoscopy. Results: The most common clinical symptoms were cough and expectoration followed by shortness of breath and hemoptysis. Chest computed tomography (CT) revealed pulmonary infection (4/11), pulmonary nodules and masses (5/11), interstitial lesions (2/11), tracheal bronchial wall thickening (5/11) or airway stenosis (4/11). Bronchoscopy showed mucosal hypertrophy (8/11), nodular bulge (3/11), and luminal stenosis (6/11). According to the lesion involvement, 1 case only involved the lungs, 10 cases involved the trachea and/or bronchus, with (8/10) or without (2/10) lung lesions. According to the bronchoscopic staging, 2 cases (2/10) in the stage Ⅰ, lesions were limited; 2 cases (2/10) in the stage Ⅱ, lesions were diffuse; 6 cases (6/10) in the stage Ⅲ, diffuse lesions with stenosis. Conclusions: PTBA is a rare disease of the respiratory system, with unspecific clinical manifestations and diverse pulmonary imaging findings; airway mucosal hypertrophy and nodular bulging can be seen under bronchoscopy. Patients with advanced stage may present with airway stenosis.