Clinical impact and prognosis of cryoglobulinemia and cryofibrinogenemia in systemic sclerosis

Chaves, S. De Almeida; Puissant, Bénédicte; Porel, T.; Bories, E.; Adoue, D.; Alric, Laurent; Astudillo, L.; Huart, Antoine; Lairez, Olivier; Michaud, Martin; Ribes, David; Prévôt, Grégoire; Sailler, L.; Gaches, F.; Pugnet, G.

doi:10.1016/j.autrev.2022.103133

Cited by 2 publications

(1 citation statement)

References 36 publications

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“…In a French cohort, CG are detected in 43% of systemic sclerosis (SSc) patients. In these patients cryoglobulinemia or cryofibrinogenemia (complexes of fibrinogen and other proteins that precipitate in cold temperature) are not associated with different clinical and bio-humoral phenotype or poor outcome [32]. Even higher attention has been paid on CV in Primary Sj€ ogren syndrome (pSS), because pSS is the most frequent autoimmune disease underlying CV and cryoglobulinemia is well recognized as an independent predictor of MALT lymphoma in pSS patients [33].…”

Section: Cryoglobulinemic Vasculitis and Autoimmune Diseasesmentioning

confidence: 99%

Cryoglobulinemic vasculitis: a 2023 update

Moretti,

Ferro,

Baldini

et al. 2023

Current Opinion in Rheumatology

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Purpose of review Cryoglobulinemic vasculitis (CV) is an immune complex mediated small vessel vasculitis characterized by the presence of cryoglobulins in serum, often associated with hepatitis C infection, systemic autoimmune diseases or hematological conditions. The focus of this review is to provide an update on new insights into pathogenesis, epidemiology and therapies of infectious and noninfectious type II and type III CV. Recent findings The introduction of new antiviral drugs for treatment of hepatitis C infection implied major changes in HCV-related CV, allowing to shed new lights on CV pathogenesis and mechanisms of relapse and, therefore, to increase the relevance of autoimmune diseases in CV epidemiology. Specific B-cell clones are involved in the production of pathogenic immune complexes that leads to small-vessel vasculitis. Therefore, both antiviral treatments [direct-acting antivirals (DAAs) and oral nucleot(s)ide analogues] and targeted anti-CD20 therapies (rituximab) prove to be safe and effective options, leading to a better prognosis. Association of Sjögren syndrome and CV defines a specific phenotype of patients, characterized by severe manifestations and poor outcome. Summary Removing viral stimulation on B-cells through direct-acting antivirals and blocking B-cells proliferation and differentiation with rituximab are the goals of treatment of CV. However, further research is needed to identify prognostic factors of refractory and relapsing disease.

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Section: Cryoglobulinemic Vasculitis and Autoimmune Diseasesmentioning

confidence: 99%

Cryoglobulinemic vasculitis: a 2023 update

Moretti,

Ferro,

Baldini

et al. 2023

Current Opinion in Rheumatology

View full text Add to dashboard Cite

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Insights into the immunological description of cryoglobulins with regard to detection and characterization in Slovenian rheumatological patients

Ogrič,

Švec,

Poljšak

et al. 2023

Immunol Res

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The detection of cryoglobulins (CG) used to diagnose cryoglobulinemic vasculitis requires strict adherence to protocol, with emphasis on the preanalytical part. Our main objectives were to introduce a more sensitive and specific protocol for the detection of CG and to characterize CG in Slovenian patients diagnosed with cryoglobulinemic vasculitis, other vasculitides, connective tissue diseases or non-rheumatic diseases examined at the Department of Rheumatology (University Medical Centre Ljubljana). Samples were routinely analyzed for the presence of CG with the protocol using the Folin-Ciocalteu reagent. In the newly introduced protocol, the type of CG was determined by immunofixation on visually observed positive samples and the concentration of CG in the cryoprecipitate and rheumatoid factor (RF) activity were measured by nephelometry. RF, C3c and C4 were measured in patients` serum and a decision tree analysis was performed using all results. The agreement between negative and positive results between the two protocols was 86%. Of the 258 patient samples tested, we found 56 patients (21.7%) with positive CG (37.5% - type II, 62.5% - type III). The RF activity was observed in 21.4% of CG positive subjects. The median concentration of type II CG was significantly higher than that of type III CG (67.4 mg/L vs. 45.0 mg/L, p = 0.037). Patients with type II had lower C4 concentrations and higher RF compared to patients with type III CG. In the decision tree, C4 was the strongest predictor of cryoglobulinemia in patients. With the newly implemented protocol, we were able to improve the detection and quantification of CG in the samples of our rheumatology patients and report the results to adequately support clinicians.

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Clinical impact and prognosis of cryoglobulinemia and cryofibrinogenemia in systemic sclerosis

Cited by 2 publications

References 36 publications

Cryoglobulinemic vasculitis: a 2023 update

Cryoglobulinemic vasculitis: a 2023 update

Insights into the immunological description of cryoglobulins with regard to detection and characterization in Slovenian rheumatological patients

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