Validation of the classification criteria for cryoglobulinaemic vasculitis

Quartuccio, Luca; Isola, Miriam; Corazza, L.; Ramos‐Casals, Manuel; Retamozo, Soledad; Ragab, Gaafar; Zoheir, Mostafa Naguib; El-Menyawi, Manal A bdel Moneim; Salem, Mohamed; Sansonno, Domenico; Ferraccioli, Gianfranco; Gremese, Elisa; Tzioufas, Athanasios G.; Voulgarelis, Michael; Vassilopoulos, Dimitrios; Scarpato, Salvatore; Pipitone, Nicolò; Salvarani, Carlo; Guillevin, Loı̈c; Terrier, Benjamin; Cacoub, Patrice; Filippini, Davide; Saccardo, Francesco; Gabrielli, Armando; Fraticelli, Paolo; Sebastiani, Marco; Tomšič, Matija; Tavoni, Antonio; Mazzaro, Cesare; Pioltelli, Pietro; Nishimoto, Norihiro; Scaini, Patrizia; Zignego, Anna Linda; Ferri, Clodoveo; Monti, Giuseppe; Pietrogrande, Maurizio; Bombardieri, Stefano; Galli, Massimo; Vita, Salvatore De

doi:10.1093/rheumatology/keu271

Cited by 74 publications

(33 citation statements)

References 11 publications

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“…All patients were thoroughly examined according to validated criteria reported elsewhere [28]. Clinical features included the presence of purpura, fatigue and arthralgia, associated or not with skin ulcers.…”

Section: Methodsmentioning

confidence: 99%

Direct-acting antiviral agents in the therapy of hepatitis C virus-related mixed cryoglobulinaemia: a single-centre experience

et al. 2017

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BackgroundThe efficacy and safety of direct-acting antiviral agents (DAAs) were evaluated in a cohort of prospectively enrolled patients with hepatitis C virus (HCV)-related mixed cryoglobulinaemia (MC), an immune complex-mediated vasculitis of small and medium vessels in which the pathogenetic role of HCV has been clearly established.MethodsTwenty-two patients received DAAs. Clinical and laboratory features were recorded at baseline, every 4 weeks until the end of treatment (EoT), and 12 weeks afterwards. Primary efficacy endpoints were (a) sustained virological response 12 weeks after therapy completion (SVR12), (b) regression of symptomatology (clinical response) and (c) cryoglobulin disappearance or cryocrit reduction ≥50% (immunological response). Complete response (CR) was defined as the occurrence of all three primary endpoints; partial response (PR) was defined as the occurrence of SVR12, with or without either immunological or clinical response; and no response was defined as missing the achievement of all three endpoints.ResultsAll patients reached SVR12. Compared with basal values, mean cryocrit values were significantly decreased at EoT and SVR12. A significant reduction of alanine transaminase and a parallel increase of complement component C4 levels were also detected. Rheumatoid factor activity was significantly reduced at EoT but not at SVR12. At SVR12, a CR was established in 14 patients (63.7%) and a PR in 8 patients (36.3%). In one patient with small lymphocytic lymphoma, the tumour progressed despite viral clearance. Mild adverse events were recorded in nine patients (40.9%).ConclusionsThe response rates induced by the use of DAAs in patients with MC were remarkably higher than those previously achieved with pegylated interferon-α/ribavirin, with or without rituximab. A much longer follow-up is desirable to achieve useful information in terms of persistent viral clearance and clinical response.

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Section: Methodsmentioning

confidence: 99%

Direct-acting antiviral agents in the therapy of hepatitis C virus-related mixed cryoglobulinaemia: a single-centre experience

et al. 2017

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“…Clinical and epidemiological data are summarized in Table . Five had type II MC (IgMk‐IgG) and CV validated according to well established criteria . A sural nerve biopsy, performed at the Department of Neurology, University of Verona, was obtained from all patients.…”

Section: Methodsmentioning

confidence: 99%

HCV RNA Genomic sequences and HCV‐E2 glycoprotein in sural nerve biopsies from HCV‐infected patients with peripheral neuropathy

Russi

Sansonno

Monaco

et al. 2017

Neuropathology Appl Neurobio

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“…Fifty-eight patients with CV [median age: 59Á2 6 11Á7 years; male/female (M/F): 0Á2] were selected according to recent validated criteria [25]. The eligibility criteria were as follows: (a) no previous administration of interferons, steroids or immunosuppressive agents; (b) serum positivity for anti-HCV antibodies and circulating HCV RNA; (c) liver biopsies showing features of chronic active hepatitis; (d) serum negativity for hepatitis B virus surface antigen (HBsAg) and anti-HIV; and (e) exclusion of connective tissue diseases and malignant lymphoproliferative disorders.…”

Section: Methodsmentioning

confidence: 99%

Activation-induced cytidine deaminase in B cells of hepatits C virus-related cryoglobulinaemic vasculitis

Russi

Dammacco

Sansonno

et al. 2015

Clinical and Experimental Immunology

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SummaryImmunoglobulin variable region heavy chain (IgV H ) somatic gene diversification is instrumental in the transformation process that characterizes hepatitis C virus (HCV)-related B cell lymphoproliferative disorders. However, the extent to which activation-induced cytidine deaminase (AID), an enzyme essential for IgV gene somatic hypermutation (SHM), is active in cryoglobulinaemic vasculitis (CV) remains unclear. AID mRNA expression in the peripheral blood of 102 chronically hepatitis C virus (HCV)-infected patients (58 with and 44 without CV) and 26 healthy subjects was investigated using real-time reverse transcription-polymerase chain reaction (RT-PCR). The features of activation-induced cytidine deaminase (AID) protein and mRNA transcripts were explored in liver tissue biopsies and portal tracts isolated using laser capture microdissection. In chronically HCV-infected patients, AID mRNA expression was almost threefold higher in those with than in those without CV and sevenfold higher than in healthy subjects (median-fold: 6Á68 versus 2Á54, P 5 0Á03 and versus 0Á95, P 5 0Á0003). AID transcript levels were significantly higher in polyclonal than in clonally restricted B cell preparations in either CV or non-CV patients (median-fold, 15Á0 versus 2Á70, P 5 0Á009 and 3Á46 versus 1Á58, P 5 0Á02, respectively). AID gene expression was found to be related negatively to age and virological parameters. AID protein was found in portal tracts containing inflammatory cells that, in several instances, expressed AID mRNA transcripts. Our data indicate that the aberrant expression of AID may reflect continuous B cell activation and sustained survival signals in HCV-related CV patients.

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Validation of the classification criteria for cryoglobulinaemic vasculitis

Abstract: Classification criteria for CV were validated in a second, large, international study confirming good sensitivity and specificity in a complex systemic disease.

Cited by 74 publications

References 11 publications

Direct-acting antiviral agents in the therapy of hepatitis C virus-related mixed cryoglobulinaemia: a single-centre experience

Direct-acting antiviral agents in the therapy of hepatitis C virus-related mixed cryoglobulinaemia: a single-centre experience

HCV RNA Genomic sequences and HCV‐E2 glycoprotein in sural nerve biopsies from HCV‐infected patients with peripheral neuropathy

Activation-induced cytidine deaminase in B cells of hepatits C virus-related cryoglobulinaemic vasculitis

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