title: Evidence of follicular T-cell implication in a case of IgG4-related systemic disease with interstitial nephritis.
abstract: 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.
authors.affiliation: Service de Néphrologie et Dialyses, AP-HP, Hôpital Tenon, Paris, France.
authors.forename: Mohamad
authors.initials: M
authors.lastname: Zaidan
authors.forename: Pascale
authors.initials: P
authors.lastname: Cervera-Pierot
authors.forename: Sophie
authors.initials: S
authors.lastname: de Seigneux
authors.forename: Karine
authors.initials: K
authors.lastname: Dahan
authors.forename: Bettina
authors.initials: B
authors.lastname: Fabiani
authors.forename: Patrice
authors.initials: P
authors.lastname: Callard
authors.forename: Pierre
authors.initials: P
authors.lastname: Ronco
authors.forename: Pierre
authors.initials: P
authors.lastname: Aucouturier
mesh_terms: D000369:Aged, 80 and over; D018450:Disease Progression; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D008198:Lymph Nodes / Q000276:immunology / Q000473:pathology; D008206:Lymphatic Diseases / Q000150:complications / Q000276:immunology / Q000473:pathology*; D008297:Male; D009395:Nephritis, Interstitial / Q000150:complications / Q000276:immunology / Q000473:pathology*; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology; D012185:Retroperitoneal Fibrosis / Q000150:complications / Q000276:immunology / Q000473:pathology*; D012793:Sialadenitis / Q000150:complications / Q000276:immunology / Q000473:pathology*; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology*; D006377:T-Lymphocytes, Helper-Inducer / Q000276:immunology / Q000473:pathology*
chemical_list: D007074:Immunoglobulin G