Clinical characteristics of amyloidosis with isolated respiratory system involvement: A review of 13 cases

BACKGROUND Isolated pulmonary amyloidosis is a very rare disease. METHODS We retrospectively reviewed the records of patients with pathologically proven isolated pulmonary amyloidosis treated at our hospital from 1990 to 2011. RESULTS There were 9 males and 4 females with a mean age of 54.7 years (range, 45-72 years) and the mean course of disease was 46.5 months (range, 5 months-15 years). The most common symptoms were cough (10/13), expectoration (8/13), hemoptysis (4/13), chest tightness (12/13), dyspnea (10/13), chest pain (3/13), fever (5/13), and body weight loss (2/13). Radiological findings included tracheal stenosis (2/13), bronchial stenosis with atelectasis (5/13), pulmonary nodules (3/13), lung consolidation (1/13), and lymph node enlargement with pleural effusion (2/13). Treatments included endotracheal stenting, endoscopic resection of tracheal and bronchial lesions, lung resection, and drug therapy with glucocorticoids, antineoplastic agents, or antibiotics. Four patients died of the disease within 1 year of diagnosis, 2 died of pneumonia at 3-4 years after original treatment, and the remaining patients are alive with follow-up ranging from 3 to 15 years. CONCLUSIONS Isolated pulmonary amyloidosis is a rare disease with a relatively high mortality and its various manifestations make diagnosis challenging. Surgical resection of lesions and chemotherapy tend to be effective treatments.