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

IgG4-related kidney disease: the effects of a Rituximab-based immunosuppressive therapy. Oncotarget. 2018 Apr 20;9(30):21337-21347 Authors: Quattrocchio G, Barreca A, Demarchi A, Solfietti L, Beltrame G, Fenoglio R, Ferro M, Mesiano P, Murgia S, Del Vecchio G, Massara C, Rollino C, Roccatello D Abstract 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...
Source: Oncotarget - Category: Cancer & Oncology Tags: Oncotarget Source Type: research