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

Oncotarget
Giacomo QuattrocchioDario Roccatello

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. A repeat renal biopsy in the patients with TIN showed a dramatic decrease in interstitial pla...Continue Reading

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Citations

Dec 14, 2018·Journal of Clinical Medicine·Philipp GaucklerAndreas Kronbichler
Jun 18, 2020·BMJ : British Medical Journal·Marco LanzillottaEmanuel Della-Torre
Sep 27, 2019·Frontiers in Immunology·Celine KaegiOnur Boyman
Jan 26, 2021·Journal of Translational Autoimmunity·Annarosa FloreaniM Eric Gershwin
Apr 22, 2021·Rheumatology International·Nikhil N TarteNirupa J Patel
Nov 13, 2019·Néphrologie & thérapeutique·Aghilès HamrounFrançois Glowacki
Apr 20, 2021·Frontiers in Medicine·Riccardo CapecchiPaola Migliorini
Apr 3, 2020·La Presse médicale·Jean-Jacques BoffaDavid Buob

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Methods Mentioned

BETA
biopsy
biopsies
light microscopy
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