Pulmonary amyloidosis

Abstract

Pulmonary amyloidosis is characterized by the deposition of monoclonal immunoglobulin light chain (AL) amyloid protein locally or diffusely in lung tissue. Local amyloid deposits in the airways, produced by B-cell clones within local tissues, may cause stridor, wheeze, cough, and haemoptysis. Diffuse alveolar deposition can occur as a complication of systemic amyloidosis. Local deposits of amyloid in the larynx, trachea, or bronchi may require treatment by endoscopic interventions, with mechanical debulking by forceps resection or laser therapy. Stenting may also be used to maintain airway patency. Radiotherapy has also been deployed successfully, and in certain circumstances may be a better option with less risk of bleeding or recurrence. Systemic AL amyloidosis may be treated by chemotherapy, while diffuse alveolar amyloidosis has a poor prognosis, particularly because it is associated with cardiac and renal amyloidosis.