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Open Access 01-12-2017 | Research article

Ectopic germinal center and megalin defect in primary Sjogren syndrome with renal Fanconi syndrome

Authors: Jing Wang, Yubing Wen, Mengyu Zhou, Xiaoxiao Shi, Lanping Jiang, Mingxi Li, Yang Yu, Xuemei Li, Xuewang Li, Wen Zhang, Andrew L. Lundquist, Limeng Chen

Published in: Arthritis Research & Therapy | Issue 1/2017

Abstract

Background

This study reports the clinical and pathological features of 12 cases of primary Sjogren syndrome (pSS) with renal involvement presenting with proximal tubular dysfunction in a single center, and investigates the possible correlation of ectopic germinal center formation and megalin/cubilin down-expression.

Method

Clinical and pathological records were reviewed. Immunohistochemistry was carried out to detect megalin, cubilin, CD21 and IL-17 expression.

Results

Patients presented with different degrees of proximal renal tubule lesion and decreased estimated glomerular filtration rate (eGFR). Renal biopsy revealed tubulointerstitial nephritis, with tubular epithelial cell degeneration, tubular atrophy, interstitial inflammation and focal fibrosis. Immunohistochemistry revealed decreased expression of megalin and cubilin, two important multiligand protein receptors on the brush border of proximal tubular epithelial cells. IL-17 secreted by Th17 subtype effector T cells was diffusely detected in the renal proximal tubule, with a negative correlation of IL-17 and megalin expression. In addition, ectopic germinal centers characterized by CD21⁺ follicular dendritic cells were present in the renal interstitium. In patients with a decreased eGFR, treatment with 4 weeks of glucocorticoid therapy resulted in an improved eGFR in 75% of patients.

Conclusion

We report 12 cases of pSS characterized by Fanconi syndrome. The decreased megalin and cubilin expression may contribute to the proximal tubular reabsorption defect, possibly secondary to Th17 infiltration and formation of ectopic germinal centers.

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Title: Ectopic germinal center and megalin defect in primary Sjogren syndrome with renal Fanconi syndrome
Authors: Jing Wang
Yubing Wen
Mengyu Zhou
Xiaoxiao Shi
Lanping Jiang
Mingxi Li
Yang Yu
Xuemei Li
Xuewang Li
Wen Zhang
Andrew L. Lundquist
Limeng Chen
Publication date: 01-12-2017
Publisher: BioMed Central
Published in: Arthritis Research & Therapy / Issue 1/2017
Electronic ISSN: 1478-6362
DOI: https://doi.org/10.1186/s13075-017-1317-x

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Ectopic germinal center and megalin defect in primary Sjogren syndrome with renal Fanconi syndrome

Abstract

Background

Method

Results

Conclusion

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