Recommendations for the management of mixed cryoglobulinemia syndrome in hepatitis C virus-infected patients

Maurizio Pietrogrande, Salvatore De Vita, Anna Linda Zignego, Pietro Pioltelli, Domenico Sansonno, Salvatore Sollima, Fabiola Atzeni, Francesco Saccardo, Luca Quartuccio, Savino Bruno, Raffaele Bruno, Mauro Campanini, Marco Candela, Laura Castelnovo, Armando Gabrielli, Giovan Battista Gaeta, Piero Marson, Maria Teresa Mascia, Cesare Mazzaro, Francesco Mazzotta & 22 others Pierluigi Meroni, Carlomaurizio Montecucco, Elena Ossi, Felice Piccinino, Daniele Prati, Massimo Puoti, Piersandro Riboldi, Agostino Riva, Dario Roccatello, Evangelista Sagnelli, Patrizia Scaini, Salvatore Scarpato, Renato Sinico, Gloria Taliani, Antonio Tavoni, Eleonora Bonacci, Piero Renoldi, Davide Filippini, Piercarlo Sarzi-Puttini, Clodoveo Ferri, Giuseppe Monti, Massimo Galli

Research output: Contribution to journalArticle

141 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)444-454
Number of pages11
JournalAutoimmunity Reviews
Volume10
Issue number8
DOIs
Publication statusPublished - Jun 2011

Fingerprint

Cryoglobulinemia
Hepacivirus
Therapeutics
Blood Component Removal
Vasculitis
Glucocorticoids
Guidelines
Skin Ulcer
Emergency Treatment
Ribavirin
Liver Function Tests
Colchicine
Expert Testimony
Peripheral Nervous System Diseases
Glomerulonephritis
Drug-Related Side Effects and Adverse Reactions
Viscosity
Cyclophosphamide
Interferons
Consensus

Keywords

  • Apheresis
  • Cryoglobulinemia
  • Cyclophosphamide
  • Glucocorticoids
  • HCV
  • Mixed cryoglobulinemia syndrome
  • Pegylated interferon
  • Ribavirin
  • Rituximab

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy

Cite this

Pietrogrande, M., De Vita, S., Zignego, A. L., Pioltelli, P., Sansonno, D., Sollima, S., ... Galli, M. (2011). Recommendations for the management of mixed cryoglobulinemia syndrome in hepatitis C virus-infected patients. Autoimmunity Reviews, 10(8), 444-454. https://doi.org/10.1016/j.autrev.2011.01.008

Recommendations for the management of mixed cryoglobulinemia syndrome in hepatitis C virus-infected patients. / Pietrogrande, Maurizio; De Vita, Salvatore; Zignego, Anna Linda; Pioltelli, Pietro; Sansonno, Domenico; Sollima, Salvatore; Atzeni, Fabiola; Saccardo, Francesco; Quartuccio, Luca; Bruno, Savino; Bruno, Raffaele; Campanini, Mauro; Candela, Marco; Castelnovo, Laura; Gabrielli, Armando; Gaeta, Giovan Battista; Marson, Piero; Mascia, Maria Teresa; Mazzaro, Cesare; Mazzotta, Francesco; Meroni, Pierluigi; Montecucco, Carlomaurizio; Ossi, Elena; Piccinino, Felice; Prati, Daniele; Puoti, Massimo; Riboldi, Piersandro; Riva, Agostino; Roccatello, Dario; Sagnelli, Evangelista; Scaini, Patrizia; Scarpato, Salvatore; Sinico, Renato; Taliani, Gloria; Tavoni, Antonio; Bonacci, Eleonora; Renoldi, Piero; Filippini, Davide; Sarzi-Puttini, Piercarlo; Ferri, Clodoveo; Monti, Giuseppe; Galli, Massimo.

In: Autoimmunity Reviews, Vol. 10, No. 8, 06.2011, p. 444-454.

Research output: Contribution to journalArticle

Pietrogrande, M, De Vita, S, Zignego, AL, Pioltelli, P, Sansonno, D, Sollima, S, Atzeni, F, Saccardo, F, Quartuccio, L, Bruno, S, Bruno, R, Campanini, M, Candela, M, Castelnovo, L, Gabrielli, A, Gaeta, GB, Marson, P, Mascia, MT, Mazzaro, C, Mazzotta, F, Meroni, P, Montecucco, C, Ossi, E, Piccinino, F, Prati, D, Puoti, M, Riboldi, P, Riva, A, Roccatello, D, Sagnelli, E, Scaini, P, Scarpato, S, Sinico, R, Taliani, G, Tavoni, A, Bonacci, E, Renoldi, P, Filippini, D, Sarzi-Puttini, P, Ferri, C, Monti, G & Galli, M 2011, 'Recommendations for the management of mixed cryoglobulinemia syndrome in hepatitis C virus-infected patients', Autoimmunity Reviews, vol. 10, no. 8, pp. 444-454. https://doi.org/10.1016/j.autrev.2011.01.008
Pietrogrande, Maurizio ; De Vita, Salvatore ; Zignego, Anna Linda ; Pioltelli, Pietro ; Sansonno, Domenico ; Sollima, Salvatore ; Atzeni, Fabiola ; Saccardo, Francesco ; Quartuccio, Luca ; Bruno, Savino ; Bruno, Raffaele ; Campanini, Mauro ; Candela, Marco ; Castelnovo, Laura ; Gabrielli, Armando ; Gaeta, Giovan Battista ; Marson, Piero ; Mascia, Maria Teresa ; Mazzaro, Cesare ; Mazzotta, Francesco ; Meroni, Pierluigi ; Montecucco, Carlomaurizio ; Ossi, Elena ; Piccinino, Felice ; Prati, Daniele ; Puoti, Massimo ; Riboldi, Piersandro ; Riva, Agostino ; Roccatello, Dario ; Sagnelli, Evangelista ; Scaini, Patrizia ; Scarpato, Salvatore ; Sinico, Renato ; Taliani, Gloria ; Tavoni, Antonio ; Bonacci, Eleonora ; Renoldi, Piero ; Filippini, Davide ; Sarzi-Puttini, Piercarlo ; Ferri, Clodoveo ; Monti, Giuseppe ; Galli, Massimo. / Recommendations for the management of mixed cryoglobulinemia syndrome in hepatitis C virus-infected patients. In: Autoimmunity Reviews. 2011 ; Vol. 10, No. 8. pp. 444-454.
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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.

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KW - Cryoglobulinemia

KW - Cyclophosphamide

KW - Glucocorticoids

KW - HCV

KW - Mixed cryoglobulinemia syndrome

KW - Pegylated interferon

KW - Ribavirin

KW - Rituximab

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