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Amyloid-associated cystic lung disease in primary Sjögren's syndrome.
Respir Med. 2013 Apr; 107(4):616-21.RM

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+Show Affiliations

Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN 55905, USA. baqir.misbah@mayo.eduNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

23402779

Citation

Baqir, Misbah, et al. "Amyloid-associated Cystic Lung Disease in Primary Sjögren's Syndrome." Respiratory Medicine, vol. 107, no. 4, 2013, pp. 616-21.
Baqir M, Kluka EM, Aubry MC, et al. Amyloid-associated cystic lung disease in primary Sjögren's syndrome. Respir Med. 2013;107(4):616-21.
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), 616-21. https://doi.org/10.1016/j.rmed.2013.01.005
Baqir M, et al. Amyloid-associated Cystic Lung Disease in Primary Sjögren's Syndrome. Respir Med. 2013;107(4):616-21. PubMed PMID: 23402779.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Amyloid-associated cystic lung disease in primary Sjögren's syndrome. AU - Baqir,Misbah, AU - Kluka,Elizabeth M, AU - Aubry,Marie-Christine, AU - Hartman,Thomas E, AU - Yi,Eunhee S, AU - Bauer,Philippe R, AU - Ryu,Jay H, Y1 - 2013/02/10/ PY - 2012/08/20/received PY - 2012/12/20/revised PY - 2013/01/10/accepted PY - 2013/2/14/entrez PY - 2013/2/14/pubmed PY - 2013/9/24/medline SP - 616 EP - 21 JF - Respiratory medicine JO - Respir Med VL - 107 IS - 4 N2 - 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. SN - 1532-3064 UR - https://www.unboundmedicine.com/medline/citation/23402779/Amyloid_associated_cystic_lung_disease_in_primary_Sjögren's_syndrome_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0954-6111(13)00010-3 DB - PRIME DP - Unbound Medicine ER -