Non HCV-related infectious cryoglobulinemia vasculitis: Results from the French nationwide CryoVas survey and systematic review of the literature

J Autoimmun. 2015 Dec;65:74-81. doi: 10.1016/j.jaut.2015.08.008. Epub 2015 Aug 29.


In patients with infectious cryoglobulinemia vasculitis (CryoVas) in the absence of hepatitis C virus infection, data on presentation, therapeutic management and outcome are lacking. We conducted a nationwide survey that included patients with HCV-negative CryoVas. We describe here the presentation, therapeutic management and outcome of 18 patients with non-HCV infectious CryoVas and 27 additional patients identified form a systematic review of the literature. We included 18 patients, mean age 57.9±13.5 years. Infectious causes were viral infections in 8 patients [hepatitis B virus (HBV) in 4, and cytomegalovirus, Epstein Barr virus, parvovirus B19 and human immunodeficiency virus in one case each], pyogenic bacterial infection in 6 patients, parasitic infection in 2 patients, and leprosy and candidiasis in one case each. Baseline manifestations were purpura (78%), glomerulonephritis (28%), arthralgia (28%), peripheral neuropathy (22%), skin necrosis (22%), cutaneous ulcers (17%), and myalgia (11%). Cryoglobulinemia was type II in 2/3 of cases. Most cases received specific anti-infectious therapy as first-line therapy, sometimes associated with corticosteroids, achieving sustained remission in the majority of cases. Refractory or relapsing patients, frequently related to HBV infection, showed a complete remission after rituximab in addition to antiviral therapy. In contrast, corticosteroids and/or immunosuppressive agents used in the absence of anti-infectious agents were frequently associated with refractory CryoVas. Viral and pyogenic bacterial infections represent the main causes of non-HCV infectious CryoVas. Antimicrobial therapy is commonly associated with sustained remission. Immunosuppressive agents should be considered only as a second-line option in patients with refractory vasculitis.

Keywords: Bacteria; Mixed cryoglobulinemia; Parasite; Vasculitis; Viruses.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use*
  • Adult
  • Aged
  • Anti-Infective Agents / therapeutic use
  • Bacterial Infections / complications
  • Cryoglobulinemia* / diagnosis
  • Cryoglobulinemia* / drug therapy
  • Cryoglobulinemia* / microbiology
  • Cytomegalovirus Infections / complications
  • Epstein-Barr Virus Infections / complications
  • Female
  • France / epidemiology
  • Hepatitis B / complications
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Middle Aged
  • Prognosis
  • Recurrence
  • Remission Induction
  • Rituximab / therapeutic use
  • Surveys and Questionnaires
  • Systemic Vasculitis* / diagnosis
  • Systemic Vasculitis* / drug therapy
  • Systemic Vasculitis* / microbiology
  • Treatment Outcome


  • Adrenal Cortex Hormones
  • Anti-Infective Agents
  • Immunosuppressive Agents
  • Rituximab