Research Papers:

IgG4-related kidney disease: the effects of a Rituximab-based immunosuppressive therapy

Giacomo Quattrocchio _, Antonella Barreca, Andrea Demarchi, Laura Solfietti, Giulietta Beltrame, Roberta Fenoglio, Michela Ferro, Paola Mesiano, Stefano Murgia, Giulio Del Vecchio, Carlo Massara, Cristiana Rollino and Dario Roccatello

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Oncotarget. 2018; 9:21337-21347. https://doi.org/10.18632/oncotarget.25095

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Giacomo Quattrocchio1, Antonella Barreca2, Andrea Demarchi3, Laura Solfietti4, Giulietta Beltrame1, Roberta Fenoglio1, Michela Ferro1, Paola Mesiano1, Stefano Murgia1, Giulio Del Vecchio1, Carlo Massara1, Cristiana Rollino1 and Dario Roccatello1,4

1Nephrology and Dialysis Unit, San Giovanni Bosco Hospital, and University of Turin, Italy

2Division of Pathology, Department of Medical Sciences, University of Turin, Turin, Italy

3Surgical Pathology Unit, San Giovanni Bosco Hospital, Turin, Italy

4Center of Research of Immunopathology and Rare Diseases (CMID), San Giovanni Bosco Hospital, and University of Turin, Turin, Italy

Correspondence to:

Giacomo Quattrocchio, email: [email protected]

Keywords: IgG4-related disease; IgG4-related kidney disease; tubulointerstitial nephritis; retroperitoneal fibrosis; Rituximab

Received: August 03, 2017     Accepted: March 24, 2018     Published: April 20, 2018


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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