Cryoglobulinemic vasculitis (CV) or mixed cryoglobulinemic syndrome (MCS) is a systemic small-vessel vasculitis characterized by the proliferation of B-cell clones producing pathogenic immune complexes, called cryoglobulins. It is often secondary to hepatitis C virus (HCV), autoimmune diseases, and hematological malignancies. CV usually has a mild benign clinical course, but severe organ damage and life-threatening manifestations can occur. Recently, evidence in favor of rituximab (RTX), an anti-CD 20 monoclonal antibody, is emerging in CV: nevertheless, questions upon the safety of this therapeutic approach, especially in HCV patients, are still being issued and universally accepted recommendations that can help physicians in MCS treatment are lacking. A Consensus Committee provided a prioritized list of research questions to perform a systematic literature review (SLR). A search was made in Medline, Embase, and Cochrane library, updated to August 2021. Of 1227 article abstracts evaluated, 27 studies were included in the SLR, of which one SLR, 4 RCTs, and 22 observational studies. Seventeen recommendations for the management of mixed cryoglobulinemia with rituximab from the Italian Study Group of Cryoglobulinemia (GISC) were developed to give a valuable tool to the physician approaching RTX treatment in CV.

Management of mixed cryoglobulinemia with rituximab: evidence and consensus-based recommendations from the Italian Study Group of Cryoglobulinemia (GISC) / Quartuccio, Luca; Bortoluzzi, Alessandra; Scirè, Carlo Alberto; Marangoni, Antonio; Del Frate, Giulia; Treppo, Elena; Castelnovo, Laura; Saccardo, Francesco; Zani, Roberta; Candela, Marco; Fraticelli, Paolo; Mazzaro, Cesare; Renoldi, Piero; Scaini, Patrizia; Filippini, Davide Antonio; Visentini, Marcella; Scarpato, Salvatore; Giuggioli, Dilia; Mascia, Maria Teresa; Sebastiani, Marco; Zignego, Anna Linda; Lauletta, Gianfranco; Fiorilli, Massimo; Casato, Milvia; Ferri, Clodoveo; Pietrogrande, Maurizio; Pioltelli, Pietro Enrico; De Vita, Salvatore; Monti, Giuseppe; Galli, Massimo. - In: CLINICAL RHEUMATOLOGY. - ISSN 0770-3198. - 42:2(2023), pp. 359-370. [10.1007/s10067-022-06391-w]

Management of mixed cryoglobulinemia with rituximab: evidence and consensus-based recommendations from the Italian Study Group of Cryoglobulinemia (GISC)

Giuggioli, Dilia;Mascia, Maria Teresa;Sebastiani, Marco;Ferri, Clodoveo;
2023

Abstract

Cryoglobulinemic vasculitis (CV) or mixed cryoglobulinemic syndrome (MCS) is a systemic small-vessel vasculitis characterized by the proliferation of B-cell clones producing pathogenic immune complexes, called cryoglobulins. It is often secondary to hepatitis C virus (HCV), autoimmune diseases, and hematological malignancies. CV usually has a mild benign clinical course, but severe organ damage and life-threatening manifestations can occur. Recently, evidence in favor of rituximab (RTX), an anti-CD 20 monoclonal antibody, is emerging in CV: nevertheless, questions upon the safety of this therapeutic approach, especially in HCV patients, are still being issued and universally accepted recommendations that can help physicians in MCS treatment are lacking. A Consensus Committee provided a prioritized list of research questions to perform a systematic literature review (SLR). A search was made in Medline, Embase, and Cochrane library, updated to August 2021. Of 1227 article abstracts evaluated, 27 studies were included in the SLR, of which one SLR, 4 RCTs, and 22 observational studies. Seventeen recommendations for the management of mixed cryoglobulinemia with rituximab from the Italian Study Group of Cryoglobulinemia (GISC) were developed to give a valuable tool to the physician approaching RTX treatment in CV.
2023
28-set-2022
42
2
359
370
Management of mixed cryoglobulinemia with rituximab: evidence and consensus-based recommendations from the Italian Study Group of Cryoglobulinemia (GISC) / Quartuccio, Luca; Bortoluzzi, Alessandra; Scirè, Carlo Alberto; Marangoni, Antonio; Del Frate, Giulia; Treppo, Elena; Castelnovo, Laura; Saccardo, Francesco; Zani, Roberta; Candela, Marco; Fraticelli, Paolo; Mazzaro, Cesare; Renoldi, Piero; Scaini, Patrizia; Filippini, Davide Antonio; Visentini, Marcella; Scarpato, Salvatore; Giuggioli, Dilia; Mascia, Maria Teresa; Sebastiani, Marco; Zignego, Anna Linda; Lauletta, Gianfranco; Fiorilli, Massimo; Casato, Milvia; Ferri, Clodoveo; Pietrogrande, Maurizio; Pioltelli, Pietro Enrico; De Vita, Salvatore; Monti, Giuseppe; Galli, Massimo. - In: CLINICAL RHEUMATOLOGY. - ISSN 0770-3198. - 42:2(2023), pp. 359-370. [10.1007/s10067-022-06391-w]
Quartuccio, Luca; Bortoluzzi, Alessandra; Scirè, Carlo Alberto; Marangoni, Antonio; Del Frate, Giulia; Treppo, Elena; Castelnovo, Laura; Saccardo, Fra...espandi
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