Analysis on the clinical and imaging features of primary tracheobronchial amyloidosis

Abstract

Objective To explore the clinical features, imaging features and treatment of primary tracheobronchial amyloidosis (PTBA). Methods The clinical data of 3 patients with PTBA who received treatment in our hospital from January 2013 to June 2017 was analyzed retrospectively combined with literature. Results In all 3 cases, 2 cases were male and 1 case was femal.The median age was 51 years old.Substantial type nodular amyloidosis was found in 1 case, tracheal bronchus amyloidosis was found in 1 case.All patients were characterized by cough, sputum, blood in phlegm, intermittent hemoptysis.Pulmonary function test showed moderate and severe obstructive ventilation dysfunction.Pulmonary interstitial diffuse amyloidosis was found in 1 case, which was characterized by chest short breath, blood in phlegm, and lung function test showed restrictive ventilatory dysfunction.The airway lumens of all 3 cases were characterized by different degree of stenosis, airway wall was uneven, annular thickening, calcification, higher density, calcification of bronchus was heavier than that of trachea.Bronchoscopy showed bronchial mucosal was hyperemia and swelling, rough, uneven, varying degrees of stricture, or occlusion.Substantial type nodular amyloidosis had good prognosis after small block clamp through bronchoscope, tracheal bronchus amyloidosis and pulmonary interstitial diffuse amyloidosis achieve uncertain efficacy and poor prognosis after treatment of lesion resection, symptomatic treatment such as prednisone+ anti-inflammatory treatment. Conclusions There was no specific clinical and imaging features of PTBA, its diagnosis depends on pathological examination.The possibility of PTBA should be considered in patients with calcification of airway wall show by CT, perfect bronchoscopy and pathology in a timely manner, so as to improve the diagnostic rate, and according to the condition to give corresponding treatment and long-term follow-up. Key words: Amyloidosis; Signs and sympoms; Tomography, X-ray computed; Treatment