Abstract
Background: Cases of amyloidosis -cystic lung lesions have been described but the association is not well understood. Most common amyloid found in the lung is AL type with deposition of protein derived from immunoglobulin light chains
Methods: We identified patients seen at Mayo Clinic, Rochester MN, USA from 1997-2011 who had biopsy proven amyloidosis and cystic lung disease by chest CT. Demographic, clinical, laboratory, pulmonary function (PFT) and pathology results were extracted
Results: Twenty-one patients (13F/8M) were identified. Median age was 61 y/o, 11 had underlying connective tissue diseases (8 Sjögren's syndrome, 1 rheumatoid arthritis, 1 overlap syndrome, 1 inclusion body myositis). PFT were available in 19 patients and revealed normal results in 8, obstructive pattern in 6, isolated reduction in diffusing capacity in 2, restrictive pattern in 1,mixed in 1, and a nonspecific pattern in 1. Thirteen of 14 patients for whom the type of amyloid could be identified had AL amyloid. Pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma was diagnosed in 5 patients; 3 of these patients had Sjögren's syndrome. In chest CT, the number of cystic lesions in the lung ranged from 1 to >10; 14 patients had >10 cysts with scattered distribution. Seventeen patients also had pulmonary nodules (>10 nodules in 10 patients); in 14 patients at least one of the pulmonary nodules were calcified
Conclusion: Amyloid–associated cystic lung disease is usually associated with AL amyloidosis and can occur in patients with or without underlying connective tissue disease (especially Sjögren's syndrome). Cystic lesion in the lung commonly >10 and are frequently associated with nodular lesions which are often calcified.
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