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

ABSTRACT

Isolated pulmonary amyloidosis is a very rare disease. We retrospectively reviewed the records of patients with pathologically proven isolated pulmonary amyloidosis treated at our hospital from 1990 to 2011. 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. 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