Managing refractory cryoglobulinemic vasculitis: challenges and solutions

Managing refractory cryoglobulinemic vasculitis: challenges and solutions

Predrag Ostojic, Ivan R Jeremic Institute of Rheumatology, School of Medicine, University of Belgrade, Belgrade, Serbia 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,...

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Autores principales: Ostojic P, Jeremic IR
Formato: article
Lenguaje:EN
Publicado: Dove Medical Press 2017
Materias:
cryoglobulinemia
vasculitis
treatment
Pathology
RB1-214
Therapeutics. Pharmacology
RM1-950
Acceso en línea:https://doaj.org/article/6c5b4364954e4cbdb2afdd21724edaa0
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spelling oai:doaj.org-article:6c5b4364954e4cbdb2afdd21724edaa02021-12-02T00:30:30ZManaging refractory cryoglobulinemic vasculitis: challenges and solutions1178-7031https://doaj.org/article/6c5b4364954e4cbdb2afdd21724edaa02017-05-01T00:00:00Zhttps://www.dovepress.com/managing-refractory-cryoglobulinemic-vasculitis-challenges-and-solutio-peer-reviewed-article-JIRhttps://doaj.org/toc/1178-7031Predrag Ostojic, Ivan R Jeremic Institute of Rheumatology, School of Medicine, University of Belgrade, Belgrade, Serbia 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, vasculitis, treatmentOstojic PJeremic IRDove Medical PressarticlecryoglobulinemiavasculitistreatmentPathologyRB1-214Therapeutics. PharmacologyRM1-950ENJournal of Inflammation Research, Vol Volume 10, Pp 49-54 (2017)
institution DOAJ
collection DOAJ
language EN
topic cryoglobulinemia
vasculitis
treatment
Pathology
RB1-214
Therapeutics. Pharmacology
RM1-950
spellingShingle cryoglobulinemia
vasculitis
treatment
Pathology
RB1-214
Therapeutics. Pharmacology
RM1-950
Ostojic P
Jeremic IR
Managing refractory cryoglobulinemic vasculitis: challenges and solutions
description Predrag Ostojic, Ivan R Jeremic Institute of Rheumatology, School of Medicine, University of Belgrade, Belgrade, Serbia 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, vasculitis, treatment
format article
author Ostojic P
Jeremic IR
author_facet Ostojic P
Jeremic IR
author_sort Ostojic P
title Managing refractory cryoglobulinemic vasculitis: challenges and solutions
title_short Managing refractory cryoglobulinemic vasculitis: challenges and solutions
title_full Managing refractory cryoglobulinemic vasculitis: challenges and solutions
title_fullStr Managing refractory cryoglobulinemic vasculitis: challenges and solutions
title_full_unstemmed Managing refractory cryoglobulinemic vasculitis: challenges and solutions
title_sort managing refractory cryoglobulinemic vasculitis: challenges and solutions
publisher Dove Medical Press
publishDate 2017
url https://doaj.org/article/6c5b4364954e4cbdb2afdd21724edaa0
work_keys_str_mv AT ostojicp managingrefractorycryoglobulinemicvasculitischallengesandsolutions
AT jeremicir managingrefractorycryoglobulinemicvasculitischallengesandsolutions
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