Primary diffuse tracheobronchial amyloidosis.

The case is reported of a woman who died at the age of 36 years from obstructive respiratory failure due to diffuse tracheobronchial amyloidosis which had caused symptoms for six years. When first seen her symptoms of wheezing cough and mucopurulent sputum sometimes streaked with blood were of recent onset, but on bronchogram and bronchoscopy her disease was already widespread. Appearances at bronchography were interpreted as malformation and at bronchoscopy as tracheobronchitis, possibly tuberculous. Bronchoscopic biopsy was necessary to make the diagnosis of amyloidosis. The amyloid gave the usual staining reactions with an apple-green birefringence in polarized light following staining with Congo red. At necropsy an extensive histological survey proved that the amyloid was confined to the trachea and main stem bronchi. There was no associated disease, no family history, and no upset in plasma proteins.