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IgG4-related kidney disease: the effects of a Rituximab-based immunosuppressive therapy.
Quattrocchio, Giacomo; Barreca, Antonella; Demarchi, Andrea; Solfietti, Laura; Beltrame, Giulietta; Fenoglio, Roberta; Ferro, Michela; Mesiano, Paola; Murgia, Stefano; Del Vecchio, Giulio; Massara, Carlo; Rollino, Cristiana; Roccatello, Dario.
Afiliación
  • Quattrocchio G; Nephrology and Dialysis Unit, San Giovanni Bosco Hospital, and University of Turin, Italy.
  • Barreca A; Division of Pathology, Department of Medical Sciences, University of Turin, Turin, Italy.
  • Demarchi A; Surgical Pathology Unit, San Giovanni Bosco Hospital, Turin, Italy.
  • Solfietti L; Center of Research of Immunopathology and Rare Diseases (CMID), San Giovanni Bosco Hospital, and University of Turin, Turin, Italy.
  • Beltrame G; Nephrology and Dialysis Unit, San Giovanni Bosco Hospital, and University of Turin, Italy.
  • Fenoglio R; Nephrology and Dialysis Unit, San Giovanni Bosco Hospital, and University of Turin, Italy.
  • Ferro M; Nephrology and Dialysis Unit, San Giovanni Bosco Hospital, and University of Turin, Italy.
  • Mesiano P; Nephrology and Dialysis Unit, San Giovanni Bosco Hospital, and University of Turin, Italy.
  • Murgia S; Nephrology and Dialysis Unit, San Giovanni Bosco Hospital, and University of Turin, Italy.
  • Del Vecchio G; Nephrology and Dialysis Unit, San Giovanni Bosco Hospital, and University of Turin, Italy.
  • Massara C; Nephrology and Dialysis Unit, San Giovanni Bosco Hospital, and University of Turin, Italy.
  • Rollino C; Nephrology and Dialysis Unit, San Giovanni Bosco Hospital, and University of Turin, Italy.
  • Roccatello D; Nephrology and Dialysis Unit, San Giovanni Bosco Hospital, and University of Turin, Italy.
Oncotarget ; 9(30): 21337-21347, 2018 Apr 20.
Article en En | MEDLINE | ID: mdl-29765543
IgG4-related disease (IgG4-RD) is a recently recognized disorder, characterized by elevated serum IgG4 concentrations, dense tissue infiltration of IgG4-positive plasma cells and storiform fibrosis. Treatment is usually based on steroids, however, relapses and long-term adverse effects are frequent. We prospectively studied 5 consecutive patients with histologically-proven IgG4-RD and renal involvement, treated with an extended Rituximab protocol combined with steroids. Two doses of intravenous cyclophosphamide were added in 4 patients. Five patients with IgG-RD were investigated: three had tubulointerstitial nephritis (TIN), while two had retroperitoneal fibrosis (RPF). In the patients with TIN, renal biospy was repeated after 1 year. In the patients with TIN, estimated glomerular filtration rate (eGFR) at 12 months increased from 9 to 24 ml/min per 1.73 m2; IgG/IgG4 decreased from 3,236/665 to 706/51 mg/dl; C3/C4 increased from 49/6 to 99/27 mg/dl; CD20+ B-cells decreased from 8.7% to 0.5%; Regulatory T-cells decreased from 7.2% to 2.5%. These functional and immunologic changes persisted at 24 months and in two patients at 36 months. A repeat renal biopsy in the patients with TIN showed a dramatic decrease in interstitial plasma cell infiltrate with normalization of IgG4/IgG positive plasma cells. The patients with RPF showed a huge regression of retroperitoneal tissue. In this sample of patients with aggressive IgG4-RD and renal involvement, treatment aimed at depleting B cells and decreasing antibody and cytokine production was associated with a substantial, persistent increase in eGFR, and a definite improvement in immunologic, radiologic and histological parameters.
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Texto completo: 1 Base de datos: MEDLINE Tipo de estudio: Guideline Idioma: En Revista: Oncotarget Año: 2018 Tipo del documento: Article

Texto completo: 1 Base de datos: MEDLINE Tipo de estudio: Guideline Idioma: En Revista: Oncotarget Año: 2018 Tipo del documento: Article