Nodular pulmonary amyloidosis with extrapulmonary involvement

Although approximately 50 cases of nodular pulmonary amyloidosis have been described in the literature, few of them have involved bilateral confluent disease. An exceptional case with disease extensive enough to cause death by respiratory insufficiency is reported. A remarkable degree of calcification in the subpleural areas, with osseous and chondroid metaplasia, characterized the amyloid deposits. Amyloid was confined to the respiratory tract except for contiguous areas (vertebral body cortex, epicardial fat, and hilar lymph nodes). A review and update of the literature since 1974 is included.