Journal
NEPHROLOGY DIALYSIS TRANSPLANTATION
Volume 26, Issue 6, Pages 2047-2050Publisher
OXFORD UNIV PRESS
DOI: 10.1093/ndt/gfr097
Keywords
follicular helper T cells; IgG4-related systemic disease; IgG subclasses; tubulointerstitial nephritis
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Funding
- Assistance Publique - Hopitaux de Paris (AP-HP)
- Institut National de la Sante Et de la Recherche Medicale (INSERM)
- Universite Pierre et Marie Curie (UPMC)
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IgG4-related systemic disease is a protean disorder that covers a wide variety of lesions. We report on a patient with tubulointerstitial nephritis, lymphadenopathies, sialadenitis and retroperitoneal fibrosis. The salivary gland and kidney interstitium were infiltrated with B lymphocytes and T lymphocytes and IgG3(+) and IgG4(+) plasma cells. The overexpression of IgG1 and IgG3, in addition to IgG4, the unusual abundance of interfollicular plasma cells and CD4(+) T cells in germinal centres of lymph nodes, and the dramatic response to rituximab point to possible roles of follicular helper T cells in enhancing a skewed B-cell terminal maturation and of CD20(+) B cells in disease progression.
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