Massimo Galli1, Giuseppe Monti2, Piero Marson3, Patrizia Scaini4, Maurizio Pietrogrande5, Marco Candela6, Laura Castelnovo7, Paola Faggioli7, Paola Novati2, Roberta Zani4, Maria Teresa Mascia8, Francesco Saccardo2, Cesare Mazzaro9, Piercarlo Sarzi-Puttini10, Marco Sebastiani11, Luca Quartuccio12, Salvatore De Vita12. 1. Infectious Diseases Unit, L. Sacco Department of Biomedical and Clinical Sciences (DIBIC), University of Milan, Milan, Italy. Electronic address: massimo.galli@unimi.it. 2. Internal Medicine Unit, Saronno Hospital, Saronno, Varese, Italy. 3. Apheresis Unit, Department of Transfusion Medicine, University Hospital of Padua, Padua, Italy. 4. Unit of Nephrology, ASST degli Spedali Civili di Brescia, Brescia, Italy. 5. Department of Health Sciences,University of Milan, Milan, Italy. 6. Internal Medicine Unit, Jesi Hospital, Jesi, Ancona, Italy. 7. Internal Medicine Unit, Legnano Hospital ASST Ovest Milanese, Legnano, Milan, Italy. 8. Rheumatology Unit, Department of Medical and Surgical Sciences for Children and Adults, Polyclinic Hospital, Modena and Reggio Emilia University, Modena, Italy. 9. Clinical of Experimental Onco-Haematology Unit, CRO Aviano National Cancer Institute IRCCS, Aviano, Pordenone, Italy. 10. Rheumatology Unit, University-Hospital Luigi Sacco, Milan, Italy. 11. Department of Rheumatology, University of Modena & Reggio Emilia, Modena, Italy. 12. Clinic of Rheumatology, Department of Medical Area (DAME), University - Hospital " Santa Maria della Misericordia", Udine, Italy.
Abstract
OBJECTIVE: Some of the manifestations of mixed cryoglobulinemia syndrome (MCS) can be severe or life-threatening, and should be rapidly contained but, as the therapeutic approaches to such conditions are largely based on anecdotal data, a consensus conference was organised by the Italian Group for the Study of Cryoglobulinemia (GISC) with the aim of providing a set of recommendations based on an in-depth survey of the available data and expert opinion. METHODS: The consensus panel, which included specialists working in different medical fields involved in the management of MCS patients, was first asked to divide the manifestations of MCS into severe or life-threatening conditions on the basis of their own experience, after which a complete literature review was carried out in accordance with the Cochrane guidelines for systematic reviews. RESULTS: Therapeutic plasma exchange (TPE) was considered the elective first-line treatment in the case of life-threatening manifestations of MCS (LT-MCS) and patients with severe clinical symptoms (S-MCS) who fail to respond to (or who are ineligible for) other treatments. The data supporting the combined use of cyclophosphamide and TPE were considered limited and inconclusive. High-dose pulsed glucocorticoid (GCS) therapy can be considered the first-line treatment of severe MCS, generally in association with TPE. Rituximab (RTX)-based treatments should be considered in patients with skin ulcers, peripheral neuropathy or glomerulonephritis, and in patients with persistent LT-MCS after TPE. In patients with hepatitis C virus-related MCS with S-MCS, viral eradication should be attempted as soon as a patient's condition allows the use of direct-acting antivirals.
OBJECTIVE: Some of the manifestations of mixed cryoglobulinemia syndrome (MCS) can be severe or life-threatening, and should be rapidly contained but, as the therapeutic approaches to such conditions are largely based on anecdotal data, a consensus conference was organised by the Italian Group for the Study of Cryoglobulinemia (GISC) with the aim of providing a set of recommendations based on an in-depth survey of the available data and expert opinion. METHODS: The consensus panel, which included specialists working in different medical fields involved in the management of MCS patients, was first asked to divide the manifestations of MCS into severe or life-threatening conditions on the basis of their own experience, after which a complete literature review was carried out in accordance with the Cochrane guidelines for systematic reviews. RESULTS: Therapeutic plasma exchange (TPE) was considered the elective first-line treatment in the case of life-threatening manifestations of MCS (LT-MCS) and patients with severe clinical symptoms (S-MCS) who fail to respond to (or who are ineligible for) other treatments. The data supporting the combined use of cyclophosphamide and TPE were considered limited and inconclusive. High-dose pulsed glucocorticoid (GCS) therapy can be considered the first-line treatment of severe MCS, generally in association with TPE. Rituximab (RTX)-based treatments should be considered in patients with skin ulcers, peripheral neuropathy or glomerulonephritis, and in patients with persistent LT-MCS after TPE. In patients with hepatitis C virus-related MCS with S-MCS, viral eradication should be attempted as soon as a patient's condition allows the use of direct-acting antivirals.
Authors: Caterina Vacchi; Marcella Visentini; Laura Gragnani; Paolo Fraticelli; Antonio Tavoni; Davide Filippini; Francesco Saccardo; Gianfranco Lauletta; Stefania Colantuono; Fabiola Atzeni; Pietro Pioltelli; Andreina Manfredi; Milvia Casato; Anna Linda Zignego; Giuseppe Monti; Maurizio Pietrogrande; Massimo Galli; Marco Sebastiani Journal: Intern Emerg Med Date: 2020-06-10 Impact factor: 3.397
Authors: Luca Quartuccio; Alessandra Bortoluzzi; Carlo Alberto Scirè; Antonio Marangoni; Giulia Del Frate; Elena Treppo; Laura Castelnovo; Francesco Saccardo; Roberta Zani; Marco Candela; Paolo Fraticelli; Cesare Mazzaro; Piero Renoldi; Patrizia Scaini; Davide Antonio Filippini; Marcella Visentini; Salvatore Scarpato; Dilia Giuggioli; Maria Teresa Mascia; Marco Sebastiani; Anna Linda Zignego; Gianfranco Lauletta; Massimo Fiorilli; Milvia Casato; Clodoveo Ferri; Maurizio Pietrogrande; Pietro Enrico Pioltelli; Salvatore De Vita; Giuseppe Monti; Massimo Galli Journal: Clin Rheumatol Date: 2022-09-28 Impact factor: 3.650
Authors: Cesare Mazzaro; Luigino Dal Maso; Laura Gragnani; Marcella Visentini; Francesco Saccardo; Davide Filippini; Pietro Andreone; Anna Linda Zignego; Valter Gattei; Giuseppe Monti; Massimo Galli; Luca Quartuccio Journal: Viruses Date: 2021-05-30 Impact factor: 5.048