Clinical analysis of primary tracheobronchial amyloidosis / 第二军医大学学报

ABSTRACT

Objective To discuss the clinical features, diagnosis, treatment and prognosis of primary tracheobronchial amyloidosis. Methods We retrospectively studied 50 primary tracheobronchial amyloidosis cases reported in Chinese literature during 1989-2012 and one in our hospital. The clinical information, symptoms, imaging findings, endoscopic manifestations and treatment were analyzed. Results The patient in our hospital was admitted due to "short breath". Chest CT, bronchoscopy and pulmonary funetion examination were done; bronchoscopy biopsy specimen confirmed the diagnosis of PTA; and the patients responded to cyclophosphamide treatment. The main clinical symptoms of the 50 patients included cough (96%, 48/ 50), short breath (70%, 35/50), sputum (66%, 33/50), fever (24%, 12/50), and hemoptysis (22%, 11/50). Thiekening of lung markings (45. 5 %, 15/33) was themost common ehest X-ray finding; airway wall thiekening and stenosis (76. 2%, 32/42) was the most common CT manifestation; and the most common endoscopic finding was also airway wall thiekening and stenosis (40. 8%, 20/49), followed by mucosal congestion and edema (32. 7%, 16/49). Obstructive ventilatory dysfunetion was found to be the major problem during pulmonary funetion tests. The diagnoses in 47(94%) patients were confirmedby bronchoscopic biopsy. Twenty-one literatures reported complications in 3 cases and misdiagnosis in 24 cases. Forty-two of the 50 patients reeeived drug treatment, bronchoscopic interventional therapy, external beam radiation therapy and surgieal treatment, and they responded well. Conclusion Primary tracheobronchial amyloidosis is rare and is prone to be misdiagnosed. Correct diagnose needs pathological examination and currently there is no specific treatment for it.