title: Amyloid-associated cystic lung disease in primary Sjögren's syndrome.
abstract: BACKGROUND
  Cystic lung disease can be seen in patients with Sjögren's syndrome (SS) and is generally thought to be due to lymphocytic interstitial pneumonia.
  
  
  METHODS
  Using 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.
  
  
  RESULTS
  Eight 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 (18)F-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.
  
  
  CONCLUSIONS
  We 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.
authors: Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN 55905, USA. baqir.misbah@mayo.edu
authors: Misbah
authors: M
authors: Baqir
authors: Elizabeth M
authors: EM
authors: Kluka
authors: Marie-Christine
authors: MC
authors: Aubry
authors: Thomas E
authors: TE
authors: Hartman
authors: Eunhee S
authors: ES
authors: Yi
authors: Philippe R
authors: PR
authors: Bauer
authors: Jay H
authors: JH
authors: Ryu
mesh_terms: D000328:Adult; D000368:Aged; D000686:Amyloidosis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D003560:Cysts / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000209:etiology; D018442:Lymphoma, B-Cell, Marginal Zone / Q000000981:diagnostic imaging / Q000209:etiology; D008297:Male; D008875:Middle Aged; D012859:Sjogren's Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D014057:Tomography, X-Ray Computed