Abstract

Amyloid fibrils can both complicate long-standing respiratory conditions and be deposited within the respiratory system itself. The manifestations, significance and prognosis of amyloid deposits are dependent on their aetiology and anatomical distribution. Amyloidosis is extremely heterogeneous and can be benign or life threatening. Each patient therefore requires thorough evaluation to determine the extent and significance of amyloid deposition and to ensure the optimal treatment. In most cases of localised amyloidosis, disease management is essentially supportive and involves resection or ablation of symptomatic deposits. In contrast, systemic anti-inflammatory treatment and chemotherapy can be extremely effective in patients with the systemic AA and AL forms of the disease. Encouragingly, the development of specific drug therapies to stabilise amyloid precursor proteins, interfere with amyloid fibrillogenesis and accelerate the clearance of tissue amyloid deposits are all on the horizon.