[Retrospective analysis of 11 cases of respiratory amyloidosis]

To investigate the clinical manifestation, diagnosis and treatment of respiratory amyloidosis.

Data of 11 patients with respiratory amyloidosis diagnosed by biopsy in Peking University First Hospital from January 2002 to January 2012 were analyzed, and the related literatures were reviewed.

In the last decade, 250 of 389 402 hospitalized patients were pathologically diagnosed as having amyloidosis, and 11 cases were pathologically confirmed to be respiratory amyloidosis. In these 11 patients, 4 cases were with serum amyloid A (AA) amyloidosis and 7 with light-chain (AL) amyloidosis. The main clinical manifestations included hoarseness, cough and dyspnea. In 4 cases with AA type unilateral larynx was involved and there was no recurrence after surgical resection. Of 7 cases with AL type, 2 cases had involvement of bilateral larynxes and both relapsed after surgery. Diffuse involvement of trachea and bronchi was found in 4 cases, and the chest CT scans showed diffuse thickening and local calcification of the airway wall, bronchial stenosis and nodules protruding into the lumen. Bronchoscopy showed airway mucosal hypertrophy, hyperemia, edema and bronchial stenosis. Lung involvement was found in 3 cases, 2 of which presented with diffuse pulmonary interstitial infiltrates, and another case presented with solitary pulmonary mass and extrapulmonary lesions. Of the 7 cases with AL type, 3 cases were treated by chemotherapy and/or radiotherapy, 3 received surgery, 2 underwent autologous hematopoietic stem cell transplantation, and 2 underwent bronchoscopic interventional therapy. Within 3 years of follow-up, 4 patients were alive, 2 dead and 1 lost to follow up.

Respiratory amyloidosis, which can be divided into AA and AL types, is clinically rare. Patients with AA type usually present with local lesions, which can be cured by surgery, while patients with AL type often present with diffuse lesions and require integrated therapies including surgery, interventional treatment, chemotherapy, radiotherapy, and autologous hematopoietic stem cell transplantation.