Corrado Campochiaro1, Emanuel Della-Torre2, Marco Lanzillotta3, Enrica Bozzolo4, Elena Baldissera5, Raffaella Milani6, Paolo Giorgio Arcidiacono7, Stefano Crippa8, Massimo Falconi9, Lorenzo Dagna10. 1. Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), Milan, Italy; Vita-Salute San Raffaele University, Milan, Italy; IRCCS San Raffaele Scientific Institute, Milan, Italy. Electronic address: campochiaro.corrado@hsr.it. 2. Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), Milan, Italy; Vita-Salute San Raffaele University, Milan, Italy; IRCCS San Raffaele Scientific Institute, Milan, Italy. Electronic address: dellatorre.emanuel@hsr.it. 3. Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), Milan, Italy; Vita-Salute San Raffaele University, Milan, Italy; IRCCS San Raffaele Scientific Institute, Milan, Italy. Electronic address: lanzillotta.marco@hsr.it. 4. Vita-Salute San Raffaele University, Milan, Italy; IRCCS San Raffaele Scientific Institute, Milan, Italy. Electronic address: bozzolo.enrica@hsr.it. 5. Vita-Salute San Raffaele University, Milan, Italy; IRCCS San Raffaele Scientific Institute, Milan, Italy. Electronic address: baldissera.elena@hsr.it. 6. Unit of Immunohematology and Transfusion Medicine, Milan, Italy; IRCCS San Raffaele Scientific Institute, Milan, Italy. Electronic address: milani.raffaella@hsr.it. 7. Pancreato-Biliary Endoscopy and Endosonography Division, Milan, Italy; IRCCS San Raffaele Scientific Institute, Milan, Italy. Electronic address: arcidiacono.paologiorgio@hsr.it. 8. Vita-Salute San Raffaele University, Milan, Italy; Division of Pancreatic Surgery, Pancreas Translational and Clinical Research Center, Milan, Italy; IRCCS San Raffaele Scientific Institute, Milan, Italy. Electronic address: crippa1.stefano@hsr.it. 9. Vita-Salute San Raffaele University, Milan, Italy; Division of Pancreatic Surgery, Pancreas Translational and Clinical Research Center, Milan, Italy; IRCCS San Raffaele Scientific Institute, Milan, Italy. Electronic address: falconi.massimo@hsr.it. 10. Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), Milan, Italy; Vita-Salute San Raffaele University, Milan, Italy; IRCCS San Raffaele Scientific Institute, Milan, Italy. Electronic address: dagna.lorenzo@hsr.it.
Abstract
BACKGROUND: IgG4-Related Disease (IgG4-RD) promptly responds to glucocorticoids but relapses in most patients. Rituximab (RTX) represents a promising strategy to avoid IgG4-RD flares but its administration for maintaining disease remission has never been assessed in terms of optimal timing of infusion, dosage, and duration of treatment. In the present study we aimed to evaluate the efficacy and safety of RTX for maintenance of IgG4-RD remission. METHODS: Fourteen patients with IgG4-RD were treated with RTX as induction of remission therapy at the San Raffaele Scientific Institute in Milan, Italy. The cohort was then divided into two study groups: patients re-treated only in case of disease relapse (Group 1, n = 7), and patients regularly re-treated with RTX every 6 months for maintenance therapy (Group 2, n = 7). Data on free-relapse rate and adverse events were collected and retrospectively analysed. RESULTS: Median follow-up time and baseline clinical-serological features were similar between Group 1 and 2 (p > 0.05). The free relapse rate 18 months after induction of remission treatment was significantly lower in Group 1 (29%) than in Group 2 (100%) (p = 0.006). Infectious complications developed in 6/14 patients (3 in Group 1 and 3 in Group 2). CONCLUSION: Administration of RTX every 6 months as maintenance of remission therapy prevents IgG4-RD flares.
BACKGROUND: IgG4-Related Disease (IgG4-RD) promptly responds to glucocorticoids but relapses in most patients. Rituximab (RTX) represents a promising strategy to avoid IgG4-RD flares but its administration for maintaining disease remission has never been assessed in terms of optimal timing of infusion, dosage, and duration of treatment. In the present study we aimed to evaluate the efficacy and safety of RTX for maintenance of IgG4-RD remission. METHODS: Fourteen patients with IgG4-RD were treated with RTX as induction of remission therapy at the San Raffaele Scientific Institute in Milan, Italy. The cohort was then divided into two study groups: patients re-treated only in case of disease relapse (Group 1, n = 7), and patients regularly re-treated with RTX every 6 months for maintenance therapy (Group 2, n = 7). Data on free-relapse rate and adverse events were collected and retrospectively analysed. RESULTS: Median follow-up time and baseline clinical-serological features were similar between Group 1 and 2 (p > 0.05). The free relapse rate 18 months after induction of remission treatment was significantly lower in Group 1 (29%) than in Group 2 (100%) (p = 0.006). Infectious complications developed in 6/14 patients (3 in Group 1 and 3 in Group 2). CONCLUSION: Administration of RTX every 6 months as maintenance of remission therapy prevents IgG4-RD flares.
Authors: Riccardo Capecchi; Domenico Giannese; Diego Moriconi; Angelo G Bonadio; Federico Pratesi; Cristina Croia; Maria F Egidi; Ilaria Puxeddu; Antonio G Tavoni; Paola Migliorini Journal: Front Med (Lausanne) Date: 2021-03-31