Non-hepatitis C virus crioglobulinemic vasculitis: a case series of nine patients

Cruz, Bóris Afonso; Melo, A L. V. de; Filho, Achiles de Almeida Cruz; Gouvêa, Patricia Salomé; Soares, Cesar José Guimarães; Batlle, Viviane S.; Hybner, M L. T.; Goulart, F B.; Pádua, Paulo Madureira de

doi:10.1590/s0482-50042006000500002

Cited by 3 publications

(9 citation statements)

References 11 publications

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“…Of the 27 studies included in this review, 20 provided insights into the risk factors associated with CV relapse. Four studies investigated relapse in type 1 CV-associated MGUS and HM (25)(26)(27)(28), while the remaining twenty studies focused on relapse in mixed CV (10,(29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40). Interestingly, seven studies (14,16,(20)(21)(22)41) failed to identify any identifiable risk factors for relapse.…”

Section: Resultsmentioning

confidence: 99%

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Risk factors for relapse in non-infectious cryoglobulinemic vasculitis, including type I cryoglobulinemia: a systematic review

2023

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BackgroundData on non-infectious cryoglobulinemic vasculitis (NICV) is scarce, especially concerning the management of relapses, which are troublesome. We aimed to investigate risk factors for relapse in NICV.MethodsA systematic literature search of CINAHL, Embase, MEDLINE, Scopus, and the Web of Science databases was implemented until April 2023. Eligible studies included randomized control trials, observational studies, and case series with ≥4 patients. Two reviewers independently extracted data and assessed the quality of the eligible studies.ResultsA total of 3,724 articles were retrieved from a database search, with 27 studies meeting the inclusion criteria for review. Most studies (n = 23) detailed relapses, with the time to relapse varying between 1 and 80 months. The relapse rate was reported at 28% in Type I NICV and ranged from 22% to 60% in mixed NICV. Risk factors for relapse in NICV were identified based on the cryoglobulin subtype and correlated with clinical and immunological responses to varying treatment regimens. Type I NICV with an associated lymphoproliferative disorder exhibited a response-relapse pattern. Cutaneous and articular involvement and incomplete clinical and immunological responses to treatment, particularly corticosteroid monotherapy and occasionally rituximab, influence the risk of relapse in Type II and Type III NICV.ConclusionOur findings underscore the significance of attaining both clinical and immunological responses and identifying risk factors for relapse in NICV. Appropriate risk stratification for NICV patients is essential for the successful implementation of effective treatment strategies.Systematic review registrationhttps://www.crd.york.ac.uk/prospero/, identifier CRD42023408140.

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Section: Resultsmentioning

confidence: 99%

“…Sixteen studies investigated relapses in NICV (sample size n = 927) (10,18,19,23,24,(29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40) whereas the remaining seven studies (sample size n = 552) were ambiguous on risk factors for relapse (16, 17, 20-22, 41, 42) consisting of a total sample size of 1,479.…”

Section: Risk Factors For Relapse In Type 2 and Type 3 MCmentioning

confidence: 99%

Risk factors for relapse in non-infectious cryoglobulinemic vasculitis, including type I cryoglobulinemia: a systematic review

2023

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“…Patients with CryoVas not related to HCV infection, affecting skin, peripheral nervous system, and kidneys (two-third with severe renal involvement), refractory to first-line therapy with glucocorticosteroids and azathioprine, were afterward successfully treated with rituximab and retreated when neccessary 36. In another retrospective study, rituximab proved to be effective in the treatment of nephropathy caused by CryoVas (improvement in 95% of patients, complete remission in 50% of patients) and in one patient with abdominal vasculitis, which is a severe form of CryoVas 37.…”

Section: Rituximab In Cryovas Not Associated With Hcv Infectionmentioning

confidence: 99%

“… 38 Efficacy in non-HCV infection-associated CryoVas was also demonstrated in case reports. 36 , 39 One case report showed efficacy of rituximab in CryoVas associated with hepatitis B viral infection, which is a quite rare condition. 40 …”

Section: Rituximab In Cryovas Not Associated With Hcv Infectionmentioning

confidence: 99%

“…According to several studies, rituximab seems to be a safe medication in patients with CryoVas, including in those with chronic HCV infection. 29 , 31 , 34 – 36 , 40 Severe adverse effects (fulminant hepatitis in HCV liver cirrhosis, serious infections, and sepsis) were rarely noticed when applied for primary glomerulonephritis treatment. 26 , 38 , 41 , 42 Respiratory infections were slightly more frequent, as well as cytopenias, when rituximab was applied along with antiviral drugs.…”

Section: Safety Of Rituximab In Cryovasmentioning

confidence: 99%

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Managing refractory cryoglobulinemic vasculitis: challenges and solutions

Ostojić

Jeremić

2017

JIR

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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.

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Essential mixed cryoglobulinemia type III with leukocytoclastic vasculitis: remission by rituximab

Baerlecken¹,

Schmidt²

2010

Clin Rheumatol

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We report about a 39-year-old female patient with severe essential mixed cryoglobulinemia of type III with leukocytoclastic vasculitis. The patient was admitted into our hospital with mesenteric lymphangiitis, which caused enteral perforation, sepsis, and pneumonia. Cryoglobulins, cryocrit, Ig-titers, and biopsy were positive for mixed cryoglobulinemia type III. We detected no signs of hepatitis C, B, or any other infectious disease. At first, disease activity could be kept under control with high doses of glucocorticoids and multiple cyclophosphamide pulses. However, after therapy with three pulses of rituximab, steroids were stopped, and the patient has not presented any symptoms for 2 years. Therefore, we suggest that rituximab affected her disease rapidly and effectively. In conclusion, rituximab is an alternative therapy for mixed cryoglobulinemia of type III with leukocytoclastic vasculitis.

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Non-hepatitis C virus crioglobulinemic vasculitis: a case series of nine patients

Cited by 3 publications

References 11 publications

Risk factors for relapse in non-infectious cryoglobulinemic vasculitis, including type I cryoglobulinemia: a systematic review

Risk factors for relapse in non-infectious cryoglobulinemic vasculitis, including type I cryoglobulinemia: a systematic review

Managing refractory cryoglobulinemic vasculitis: challenges and solutions

Essential mixed cryoglobulinemia type III with leukocytoclastic vasculitis: remission by rituximab

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