Amyloid-associated cystic lung disease in primary Sjögren's syndrome

Misbah Baqir, Elizabeth M. Kluka, Marie Christine Aubry, Thomas E. Hartman, Eunhee S. Yi, Philippe R. Bauer, Jay H Ryu

Research output: Contribution to journalArticle

31 Citations (Scopus)

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 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. 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.

Original languageEnglish (US)
Pages (from-to)616-621
Number of pages6
JournalRespiratory Medicine
Volume107
Issue number4
DOIs
StatePublished - Apr 2013

Fingerprint

Amyloid
Lung
Amyloidosis
Marginal Zone B-Cell Lymphoma
Biopsy
Cystic Disease Of Lung
Interstitial Lung Diseases
Deoxyglucose
Cough
Positron-Emission Tomography
Dyspnea

Keywords

  • Amyloidosis
  • Cystic lung disease
  • Sjogren's syndrome

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Baqir, M., Kluka, E. M., Aubry, M. C., Hartman, T. E., Yi, E. S., Bauer, P. R., & Ryu, J. H. (2013). Amyloid-associated cystic lung disease in primary Sjögren's syndrome. Respiratory Medicine, 107(4), 616-621. https://doi.org/10.1016/j.rmed.2013.01.005

Amyloid-associated cystic lung disease in primary Sjögren's syndrome. / Baqir, Misbah; Kluka, Elizabeth M.; Aubry, Marie Christine; Hartman, Thomas E.; Yi, Eunhee S.; Bauer, Philippe R.; Ryu, Jay H.

In: Respiratory Medicine, Vol. 107, No. 4, 04.2013, p. 616-621.

Research output: Contribution to journalArticle

Baqir, M, Kluka, EM, Aubry, MC, Hartman, TE, Yi, ES, Bauer, PR & Ryu, JH 2013, 'Amyloid-associated cystic lung disease in primary Sjögren's syndrome', Respiratory Medicine, vol. 107, no. 4, pp. 616-621. https://doi.org/10.1016/j.rmed.2013.01.005
Baqir M, Kluka EM, Aubry MC, Hartman TE, Yi ES, Bauer PR et al. Amyloid-associated cystic lung disease in primary Sjögren's syndrome. Respiratory Medicine. 2013 Apr;107(4):616-621. https://doi.org/10.1016/j.rmed.2013.01.005
Baqir, Misbah ; Kluka, Elizabeth M. ; Aubry, Marie Christine ; Hartman, Thomas E. ; Yi, Eunhee S. ; Bauer, Philippe R. ; Ryu, Jay H. / Amyloid-associated cystic lung disease in primary Sjögren's syndrome. In: Respiratory Medicine. 2013 ; Vol. 107, No. 4. pp. 616-621.
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