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Ostojic P, Jeremic IR. Managing refractory cryoglobulinemic vasculitis: challenges and solutions. J Inflamm Res. 2017;10:49-54doi: 10.2147/JIR.S114067.
Ostojic, P., & Jeremic, I. R. (2017). Managing refractory cryoglobulinemic vasculitis: challenges and solutions. Journal of inflammation research, 1049-54. https://doi.org/10.2147/JIR.S114067
Ostojic, Predrag, and Jeremic, Ivan R. "Managing refractory cryoglobulinemic vasculitis: challenges and solutions." Journal of inflammation research vol. 10 (2017): 49-54. doi: https://doi.org/10.2147/JIR.S114067
Ostojic P, Jeremic IR. Managing refractory cryoglobulinemic vasculitis: challenges and solutions. J Inflamm Res. 2017 May 08;10:49-54. doi: 10.2147/JIR.S114067. eCollection 2017. PMID: 28507447; PMCID: PMC5428757.
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J Inflamm Res. 2017 May 08;10:49-54. doi: 10.2147/JIR.S114067. eCollection 2017.

Managing refractory cryoglobulinemic vasculitis: challenges and solutions.

Journal of inflammation research

Predrag Ostojic, Ivan R Jeremic

Affiliations

  1. Institute of Rheumatology, School of Medicine, University of Belgrade, Belgrade, Serbia.

PMID: 28507447 PMCID: PMC5428757 DOI: 10.2147/JIR.S114067

Abstract

Cryoglobulinemia is thought to be a rare condition. It may be an isolated disorder or secondary to a particular disease. According to immunoglobulin composition, cryoglobulinemia is classified into three types. In mixed cryoglobulinemia (types II and III), vascular deposition of cryoglobulin-containing immune complexes and complement may induce a clinical syndrome, characterized by systemic vasculitis and inflammation - cryoglobulinemic vasculitis (CryoVas). Most common clinical manifestations in CryoVas are skin lesions (orthostatic purpura and ulcers), weakness, peripheral neuropathy, Raynaud's phenomenon, sicca syndrome, membranoproliferative glomerulonephritis, and arthralgia and seldom arthritis. In patients with mixed cryoglobulinemia, prevalence of anti-hepatitis C virus (HCV) antibodies and/or HCV RNA, detected by polymerase chain reaction (PCR), is reported to be up to 90%, indicating a significant role of HCV in the development of this condition. The goals of therapy for mixed cryoglobulinemia include immunoglobulin level reduction and antigen elimination. CryoVas not associated with HCV infection should be treated according to treatment recommendations for small-vessel vasculitides. CryoVas associated with chronic HCV infection should be treated with antivirals along with immunosuppressive drugs, with or without plasmapheresis, depending on disease severity and organ involvement. Patients who do not respond to first-line therapy may achieve remission when treatment with rituximab is started as second-line therapy. In HCV-related CryoVas, antiviral therapy should be given along with rituximab in order to achieve complete or partial remission. Moreover, rituximab has proven to be a glucocorticoid-sparing medication. Other potential therapies for refractory CryoVas include mycophenolate mofetil and belimumab, while tumor necrosis factor (TNF) inhibitors are not effective.

Keywords: cryoglobulinemia; treatment; vasculitis

Conflict of interest statement

Disclosure The authors report no conflicts of interest in this work.

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