PULMONARY AMYLOIDOSIS - THE MAYO-CLINIC EXPERIENCE FROM 1980 TO 1993

Objective: To define the prognosis for and radiographic presentation o f patients with pulmonary amyloidosis. Design: Retrospective review of the Mayo Clinic experience with biopsy-proven pulmonary amyloidosis f rom 1980 to 1993. Setting: Tertiary care center. Patients: Patients wi th pulmonary biopsy specimens showing amyloid deposition.Measurements: Medical records were reviewed, and pertinent information was recorded , including demographic data, type of pulmonary biopsy, results of bio psies of nonpulmonary sites and of immunoelectrophoresis, and other cl inical, radiographic, and laboratory information necessary for disting uishing localized pulmonary amyloidosis, primary systemic amyloidosis, secondary amyloidosis, and familial amyloidosis. Results: 35 of 55 pa tients with pulmonary amyloidosis had primary systemic amyloidosis tha t presented radiographically as an interstitial or reticulonodular pat tern with or without pleural effusion. The median survival after diagn osis was 16 months. Nodular pulmonary ''amyloidomas'' (nodular amyloid lesions) were not associated with systemic disease and were associate d with a benign prognosis. Three of 4 patients with localized tracheob ronchial amyloidosis required Nd:YAC (neodymium:yttrium-aluminum-garne t) laser therapy for obstructive symptoms. ''Senile'' amyloid depositi on was an incidental finding in some patients at autopsy. Conclusions: Localized amyloidomas are characterized by a benign course and are no t associated with systemic amyloidosis. Despite its localized nature, tracheobronchial amyloid deposition may be asymptomatic or may result in significant morbidity due to obstructive phenomena. Pulmonary amylo idosis associated with primary systemic amyloidosis generally presents as a diffuse interstitial pattern with or without pleural effusion. C omplete survival data indicate that long-term outcome is poor after di agnosis. We describe the largest series of patients diagnosed by bronc hoscopic lung biopsy. Despite reports to the contrary, we have found b ronchoscopic lung biopsy to be a safe and effective diagnostic techniq ue.