TY - JOUR
T1 - Recommendations for the management of mixed cryoglobulinemia syndrome in hepatitis C virus-infected patients
AU - Pietrogrande, Maurizio
AU - De Vita, Salvatore
AU - Zignego, Anna Linda
AU - Pioltelli, Pietro
AU - Sansonno, Domenico
AU - Sollima, Salvatore
AU - Atzeni, Fabiola
AU - Saccardo, Francesco
AU - Quartuccio, Luca
AU - Bruno, Savino
AU - Bruno, Raffaele
AU - Campanini, Mauro
AU - Candela, Marco
AU - Castelnovo, Laura
AU - Gabrielli, Armando
AU - Gaeta, Giovan Battista
AU - Marson, Piero
AU - Mascia, Maria Teresa
AU - Mazzaro, Cesare
AU - Mazzotta, Francesco
AU - Meroni, Pierluigi
AU - Montecucco, Carlomaurizio
AU - Ossi, Elena
AU - Piccinino, Felice
AU - Prati, Daniele
AU - Puoti, Massimo
AU - Riboldi, Piersandro
AU - Riva, Agostino
AU - Roccatello, Dario
AU - Sagnelli, Evangelista
AU - Scaini, Patrizia
AU - Scarpato, Salvatore
AU - Sinico, Renato
AU - Taliani, Gloria
AU - Tavoni, Antonio
AU - Bonacci, Eleonora
AU - Renoldi, Piero
AU - Filippini, Davide
AU - Sarzi-Puttini, Piercarlo
AU - Ferri, Clodoveo
AU - Monti, Giuseppe
AU - Galli, Massimo
PY - 2011/6
Y1 - 2011/6
N2 - Objective: The objective of this review was to define a core set of recommendations for the treatment of HCV-associated mixed cryoglobulinemia syndrome (MCS) by combining current evidence from clinical trials and expert opinion. Methods: Expert physicians involved in studying and treating patients with MCS formulated statements after discussing the published data. Their attitudes to treatment approaches (particularly those insufficiently supported by published data) were collected before the consensus conference by means of a questionnaire, and were considered when formulating the statements. Results: An attempt at viral eradication using pegylated interferon plus ribavirin should be considered the first-line therapeutic option in patients with mild-moderate HCV-related MCS. Prolonged treatment (up to 72. weeks) may be considered in the case of virological non-responders showing clinical and laboratory improvements. Rituximab (RTX) should be considered in patients with severe vasculitis and/or skin ulcers, peripheral neuropathy or glomerulonephritis. High-dose pulsed glucocorticoid (GC) therapy is useful in severe conditions and, when necessary, can be considered in combination with RTX; on the contrary, the majority of conference participants discouraged the chronic use of low-medium GC doses. Apheresis remains the elective treatment for severe, life-threatening hyper-viscosity syndrome; its use should be limited to patients who do not respond to (or who are ineligible for) other treatments, and emergency situations. Cyclophosphamide can be considered in combination with apheresis, but the data supporting its use are scarce. Despite the limited available data, colchicine is used by many of the conference participants, particularly in patients with mild-moderate MCS refractory to other therapies. Careful monitoring of the side effects of each drug, and its effects on HCV replication and liver function tests is essential. A low-antigen-content diet can be considered as supportive treatment in all symptomatic MCS patients. Although there are no data from controlled trials, controlling pain should always be attempted by tailoring the treatment to individual patients on the basis of the guidelines used in other vasculitides. Conclusion: Although there are few controlled randomised trials of MCS treatment, increasing knowledge of its pathogenesis is opening up new frontiers. The recommendations provided may be useful as provisional guidelines for the management of MCS.
AB - Objective: The objective of this review was to define a core set of recommendations for the treatment of HCV-associated mixed cryoglobulinemia syndrome (MCS) by combining current evidence from clinical trials and expert opinion. Methods: Expert physicians involved in studying and treating patients with MCS formulated statements after discussing the published data. Their attitudes to treatment approaches (particularly those insufficiently supported by published data) were collected before the consensus conference by means of a questionnaire, and were considered when formulating the statements. Results: An attempt at viral eradication using pegylated interferon plus ribavirin should be considered the first-line therapeutic option in patients with mild-moderate HCV-related MCS. Prolonged treatment (up to 72. weeks) may be considered in the case of virological non-responders showing clinical and laboratory improvements. Rituximab (RTX) should be considered in patients with severe vasculitis and/or skin ulcers, peripheral neuropathy or glomerulonephritis. High-dose pulsed glucocorticoid (GC) therapy is useful in severe conditions and, when necessary, can be considered in combination with RTX; on the contrary, the majority of conference participants discouraged the chronic use of low-medium GC doses. Apheresis remains the elective treatment for severe, life-threatening hyper-viscosity syndrome; its use should be limited to patients who do not respond to (or who are ineligible for) other treatments, and emergency situations. Cyclophosphamide can be considered in combination with apheresis, but the data supporting its use are scarce. Despite the limited available data, colchicine is used by many of the conference participants, particularly in patients with mild-moderate MCS refractory to other therapies. Careful monitoring of the side effects of each drug, and its effects on HCV replication and liver function tests is essential. A low-antigen-content diet can be considered as supportive treatment in all symptomatic MCS patients. Although there are no data from controlled trials, controlling pain should always be attempted by tailoring the treatment to individual patients on the basis of the guidelines used in other vasculitides. Conclusion: Although there are few controlled randomised trials of MCS treatment, increasing knowledge of its pathogenesis is opening up new frontiers. The recommendations provided may be useful as provisional guidelines for the management of MCS.
KW - Apheresis
KW - Cryoglobulinemia
KW - Cyclophosphamide
KW - Glucocorticoids
KW - HCV
KW - Mixed cryoglobulinemia syndrome
KW - Pegylated interferon
KW - Ribavirin
KW - Rituximab
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U2 - 10.1016/j.autrev.2011.01.008
DO - 10.1016/j.autrev.2011.01.008
M3 - Article
C2 - 21303705
AN - SCOPUS:79957655345
VL - 10
SP - 444
EP - 454
JO - Autoimmunity Reviews
JF - Autoimmunity Reviews
SN - 1568-9972
IS - 8
ER -