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• W2077990166 abstract "BackgroundCystic lung disease can be seen in patients with Sjögren's syndrome (SS) and is generally thought to be due to lymphocytic interstitial pneumonia.MethodsUsing computer-assisted search we identified patients with primary SS seen at Mayo Clinic, Rochester, MN during a 14-year period from 1997 to 2010 who were diagnosed with pulmonary amyloidosis confirmed on lung biopsy. Clinical records, imaging studies, and pathologic specimens were reviewed to delineate presenting features, diagnostic evaluation, and clinical course.ResultsEight patients (7 women, 1 man) with primary SS were diagnosed with pulmonary amyloidosis by lung biopsy (7 surgical, 1 bronchoscopic). Their median age was 55 years (range, 32–75 years) and all were nonsmokers. Presenting symptoms included dyspnea and cough but 4 patients presented with radiologic abnormalities in the absence of respiratory symptoms. CT findings included cystic lesions and nodular opacities in all eight patients. PET scan performed in six patients did not reveal 18F-2-deoxyglucose (FDG) uptake except in one nodule with borderline uptake. Lung biopsy demonstrated the presence of amyloid in all patients and was associated with mucosa-associated lymphoid tissue (MALT) lymphoma in three patients. Pulmonary function results were normal in five patients and revealed mild impairment in a mixed pattern in one patient.ConclusionsWe conclude cystic and nodular lung lesions seen in patients with primary SS can represent amyloidosis which can be associated with MALT lymphoma in some of these patients." @default.
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• W2077990166 date "2013-04-01" @default.
• W2077990166 modified "2023-10-16" @default.
• W2077990166 title "Amyloid-associated cystic lung disease in primary Sjögren's syndrome" @default.
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• W2077990166 doi "https://doi.org/10.1016/j.rmed.2013.01.005" @default.
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