Long-term effects of intensive B cell depletion therapy in severe cases of IgG4-related disease with renal involvement
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ORIGINAL ARTICLE
Long-term effects of intensive B cell depletion therapy in severe cases of IgG4-related disease with renal involvement Giacomo Quattrocchio 1 & Antonella Barreca 2 & Andrea Demarchi 3 & Roberta Fenoglio 1 & Michela Ferro 1 & Giulio Del Vecchio 1 & Carlo Massara 1 & Cristiana Rollino 1 & Savino Sciascia 1 & Dario Roccatello 1 Received: 5 August 2020 / Accepted: 30 October 2020 # The Author(s) 2020
Abstract IgG4-related disease (IgG4-RD) is an immune-mediated disorder often showing elevated serum IgG4 concentrations, dense T and B lymphocyte infiltration, and IgG4-positive plasma cells and storiform fibrosis. We prospectively evaluated for 4 years 5 patients with histologically proven IgG4-RD of whom 3 had tubulointerstitial nephritis (TIN) and 2 had retroperitoneal fibrosis (RPF). They received an intensive B depletion therapy with rituximab. The estimated glomerular filtration rate of TIN patients after 1 year increased from 9 to 24 ml/min per 1.73 m2. IgG/IgG4 dropped from 3236/665 to 706/51 mg/dl, C3/C4 went up from 49/6 to 99/27 mg/dl, and the IgG4-RD responder index fell from 10 to 1. CD20+ B cells decreased from 8.7 to 0.5%. A striking drop in interstitial plasma cell infiltrate as well as normalization of IgG4/IgG-positive plasma cells was observed at repeat biopsy. Both clinical and immunological improvement persisted over a 4-year follow-up. Treating these patients who were affected by aggressive IgG4-RD with renal involvement in an effort to induce a prolonged B cells depletion with IgG4 and cytokine production decrease resulted in a considerable rise in eGFR, with IgG4-RD RI normalization and a noteworthy improvement in clinical and histological features. Furthermore, the TIN subgroup was shown not to need for any maintenance therapy. Keywords IgG4-related disease . IgG4-related disease with kidney involvement . Tubulointerstitial nephritis . Retroperitoneal fibrosis . Rituximab . B cell depletion therapy
Introduction Often times, IgG4-related disease (IgG4-RD) is a heterogeneous disorder, whose features may include different organ involvement, high IgG4 concentration in the serum, and unusual histological findings. In detail, the latest include IgG4+ plasma-cell-enriched dense lymphoplasmacytic infiltrate, a storiform pattern of fibrosis, and obliterative phlebitis [1, 2]. Men in their 50s and above are most often affected and generally display non-severe clinical presentation, potentially
* Giacomo Quattrocchio [email protected] 1
Nephrology and Dialysis Universitary Unit, and Center of Research of Immunopathology and Rare Diseases (CMID) San Giovanni Bosco Hospital, and Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
2
Department of Biomedical Sciences, University of Turin, Turin, Italy
3
Pathology Unit, San Giovanni Bosco Hospital, Turin, Italy
mimicking several neoplastic, infectious, and inflammatory diseases [3]. Most common laboratory features are high serum concentration of IgG4 levels (observed in 50–70% cases) (> 135 mg/ dl) of
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