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Open Access Highly Accessed Case report

EBV-positive diffuse large B-cell lymphoma in a patient with primary Sjögren’s syndrome and membranous glomerulonephritis

Chang Seong Kim1, Yoo Duk Choi2, Joon Seok Choi1, Eun Hui Bae1, Seong Kwon Ma1 and Soo Wan Kim1*

Author Affiliations

1 Department of Internal Medicine, Chonnam National University Medical School, 42 Jebongro, Gwangju, 501-757, South Korea

2 Department of pathology, Chonnam National University Medical School, Gwangju, South Korea

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BMC Nephrology 2012, 13:149  doi:10.1186/1471-2369-13-149

Published: 15 November 2012

Abstract

Background

Sjögren’s syndrome is a systemic autoimmune disease in which lymphatic cells destroy the salivary and lacrimal glands. Glomerulonephritis is thought to be a rare occurrence in primary Sjögren’s syndrome. Furthermore, concurrent glomerular involvement and lymphoma in patients with Sjögren’s syndrome has seldom been reported.

Case presentation

A 52-year-old woman with primary Sjögren’s syndrome developed membranous glomerulonephritis and Epstein-Barr virus-positive diffuse large B-cell lymphoma (DLBCL). She was diagnosed with Sjögren’s syndrome based on the dry eyes, dry mouth, positive anti-nuclear antibody test, anti-Ro (SS-A) antibody, salivary gland biopsy, and salivary scintigraphy. Moreover, renal biopsy confirmed the diagnosis of membranous glomerulonephritis. Three months later, her small bowel was perforated with pneumoperitoneum, and the biopsy revealed Epstein-Barr virus-positive DLBCL.

Conclusions

We observed the first case of primary Sjögren’s syndrome associated with Epstein-Barr Virus-positive DLBCL and membranous glomerulonephritis. Because of the possibility of malignancy-associated membranous glomerulonephritis in patients with primary Sjögren’s syndrome, we should be careful and examine such patients for hidden malignancy.

Keywords:
Primary Sjögren’s syndrome; Membranous glomerulonephritis; EBV-positive diffuse large B-cell lymphoma