Recommendations for managing the manifestations of severe and life-threatening mixed cryoglobulinemia syndrome

Galli, Massimo; Monti, Giuseppe; Marson, Piero; Scaini, Patrizia; Pietrogrande, Maurizio; Candela, Marco; Castelnovo, Laura; Faggioli, Paola; Novati, P; Zani, Roberta; Mascia, Maria Teresa; Saccardo, Francesco; Mazzaro, Cesare; Sarzi‐Puttini, Piercarlo; Sebastiani, Marco; Quartuccio, Luca; Vita, Salvatore De

doi:10.1016/j.autrev.2019.06.008

Cited by 37 publications

(34 citation statements)

References 75 publications

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“…Cyclophosphamide has been used in association with apheresis to reduce the post apheresis rebound in cryoglobulinemic production [59], but it was only used in 19.5% of the patients reported by Marson and colleagues [58], mainly in association with the first apheresis session. The cost-benefit ratio of adding cyclophosphamide to apheresis is a subject of debate, and the choice should be evaluated in each case [60]. Data from small case series support the effectiveness of pulse high-dose corticosteroid therapy in controlling CV flares [59], and corticosteroids were associated with apheresis at different times and doses in treating 86% of the patients included in the study by Marson and colleagues [58].…”

Section: Plasma Exchangementioning

confidence: 99%

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Hepatitis B Virus-Related Cryoglobulinemic Vasculitis: Review of the Literature and Long-Term Follow-Up Analysis of 18 Patients Treated with Nucleos(t)ide Analogues from the Italian Study Group of Cryoglobulinemia (GISC)

Mazzaro

Maso

Gragnani

et al. 2021

Viruses

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Hepatitis B virus (HBV) chronic infection causes progressive liver damage, although about 20% of patients develop extrahepatic manifestations such as cryoglobulinemic vasculitis (CV). Clinical manifestations range from mild to moderate (purpura, asthenia, arthralgia) to severe (leg ulcers, peripheral neuropathy, glomerulonephritis, non-Hodgkin lymphoma). A comprehensive review of therapeutic options for HBV-related CV is lacking. Nucleos(t)ide analogues (NA) suppress HBV replication in 90–100% of cases and induce clinical response in most patients with mild-to-moderate CV. Plasma exchange can be performed in patients with severe CV and should be considered in severe or life-threatening cases combined with high doses of corticosteroids and antiviral treatment. A cautious use of rituximab can be considered only in association with NA treatment in refractory cases. A review of the literature and an analysis of data collected by six centers of the Italian Group for the Study of Cryoglobulinemia on 18 HBV-CV nucleotide/nucleoside analogues (NAs)-treated patients were carried out.

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Section: Plasma Exchangementioning

confidence: 99%

“…In high doses or as pulse therapy, corticosteroids have been in use for many years in subjects with severe systemic CV (ulcer, progressive peripheral neuropathy, and glomerulonephritis) [62]. The high doses of corticosteroids, both employed alone or associated with apheresis, are recommended by GISC to control severe CV flare [60].…”

Section: Steroidsmentioning

confidence: 99%

Hepatitis B Virus-Related Cryoglobulinemic Vasculitis: Review of the Literature and Long-Term Follow-Up Analysis of 18 Patients Treated with Nucleos(t)ide Analogues from the Italian Study Group of Cryoglobulinemia (GISC)

Mazzaro

Maso

Gragnani

et al. 2021

Viruses

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“…Severe CV manifestations, such as renal involvement (membranoproliferative glomerulonephritis), skin necrosis, peripheral neuropathy, gastrointestinal vasculitis (intestinal ischemia), lung involvement, heart and/or CNS vasculitis, may require additional immunosuppressive drugs to DAA therapy (Figure 1). 42 Rituximab is discussed for HCV-CV patients with severe systemic manifestations, including renal involvement, large necrotizing ulcers, and polyneuropathy. 40,43 Rituximab is administered intravenously at a dose of 375 mg/m 2 on days 1, 8, 15, and 22.…”

Section: Immune Restoration Following Daa Therapymentioning

confidence: 99%

“…[2][3][4]6,12,13,16,17 Mixed cryoglobulinemia is detected in 40 1). 42 Rituximab is discussed for HCV-CV patients with severe systemic manifestations, including renal involvement, large necrotizing ulcers, and polyneuropathy. 40,43 Rituximab is administered intravenously at a dose of 375 mg/m 2 on days 1, 8, 15, and 22.…”

Section: Immune Restoration Following Daa Therapymentioning

confidence: 99%

Treatment of chronic hepatitis C-associated cryoglobulinemia vasculitis at the era of direct-acting antivirals

Comarmond

Cacoub

Saadoun

2020

Therap Adv Gastroenterol

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Hepatitis C virus (HCV) infection is responsible for both hepatic and extrahepatic manifestations. Before the era of direct-acting antivirals (DAA), cryoglobulinemia was related to HCV infection in 70–90% of cases. Observed in 30% to 40% of patients with hepatitis C, mixed cryoglobulinemia is mainly asymptomatic. Conversely, symptomatic cryoglobulinemia vasculitis (CV) can occur in 5–10% of patients with HCV-associated cryoglobulinemia. CV is a small-vessel systemic vasculitis, and organ damage results from circulation and precipitation of cryoglobulins and complement activation. A wide range of clinical symptoms can be observed during CV, and manifestations are potentially life-threatening. The most frequent manifestations occurring in CV are cutaneous, with recurrent purpura, articular with joint pains, neurologic with peripheric neuropathy, and renal with membranoproliferative glomerulonephritis. DAA have drastically changed chronic HCV therapy. DAA induce sustained virological response (SVR) rates greater than 95%, and also improve extrahepatic manifestations such as CV. We review recent studies investigating the clinical and immune effects of DAA therapy on HCV-CV.

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“…The therapeutic management of CV depends on the underlying trigger and the severity of disease. When polyneuropathy or kidney involvement are present, immunosuppressive treatment or plasma-exchange strategies are usually required [ 52 ].…”

Section: Introductionmentioning

confidence: 99%

Diagnosis and management of leukocytoclastic vasculitis

Fraticelli

Gabrielli

2021

Intern Emerg Med

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Leukocytoclastic vasculitis (LCV) is a histopathologic description of a common form of small vessel vasculitis (SVV), that can be found in various types of vasculitis affecting the skin and internal organs. The leading clinical presentation of LCV is palpable purpura and the diagnosis relies on histopathological examination, in which the inflammatory infiltrate is composed of neutrophils with fibrinoid necrosis and disintegration of nuclei into fragments (“leukocytoclasia”). Several medications can cause LCV, as well as infections, or malignancy. Among systemic diseases, the most frequently associated with LCV are ANCA-associated vasculitides, connective tissue diseases, cryoglobulinemic vasculitis, IgA vasculitis (formerly known as Henoch–Schonlein purpura) and hypocomplementemic urticarial vasculitis (HUV). When LCV is suspected, an extensive workout is usually necessary to determine whether the process is skin-limited, or expression of a systemic vasculitis or disease. A comprehensive history and detailed physical examination must be performed; platelet count, renal function and urinalysis, serological tests for hepatitis B and C viruses, autoantibodies (anti-nuclear antibodies and anti-neutrophil cytoplasmic antibodies), complement fractions and IgA staining in biopsy specimens are part of the usual workout of LCV. The treatment is mainly focused on symptom management, based on rest (avoiding standing or walking), low dose corticosteroids, colchicine or different unproven therapies, if skin-limited. When a medication is the cause, the prognosis is favorable and the discontinuation of the culprit drug is usually resolutive. Conversely, when a systemic vasculitis is the cause of LCV, higher doses of corticosteroids or immunosuppressive agents are required, according to the severity of organ involvement and the underlying associated disease.

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Recommendations for managing the manifestations of severe and life-threatening mixed cryoglobulinemia syndrome

Cited by 37 publications

References 75 publications

Hepatitis B Virus-Related Cryoglobulinemic Vasculitis: Review of the Literature and Long-Term Follow-Up Analysis of 18 Patients Treated with Nucleos(t)ide Analogues from the Italian Study Group of Cryoglobulinemia (GISC)

Hepatitis B Virus-Related Cryoglobulinemic Vasculitis: Review of the Literature and Long-Term Follow-Up Analysis of 18 Patients Treated with Nucleos(t)ide Analogues from the Italian Study Group of Cryoglobulinemia (GISC)

Treatment of chronic hepatitis C-associated cryoglobulinemia vasculitis at the era of direct-acting antivirals

Diagnosis and management of leukocytoclastic vasculitis

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