Prevalence of mixed cryoglobulinaemia syndrome and circulating cryoglobulins in a population-based survey: the Origgio study

Monti, Giuseppe; Saccardo, Francesco; Castelnovo, Laura; Novati, P; Sollima, Salvatore; Riva, Agostino; Sarzi-Puttini, Piercarlo; Quartuccio, Luca; Vita, Salvatore De; Galli, Massimo

doi:10.1016/j.autrev.2013.11.005

Cited by 39 publications

(15 citation statements)

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“…It has been frequently reported that HCV infection is associated with mixed cryoglobulins but not with type I cryoglobulins . Our results were consistent with these findings, with nearly 50% of the tested patients with mixed cryoglobulins being HCV‐positive (in contrast with HBV or HIV infections, which were rare).…”

Section: Discussionsupporting

confidence: 91%

“…The characteristics of cryoglobulins reported in the literature based on type, distribution, and prevalence of underlying pathology vary between studies, due to population selection bias and different cryoglobulin detection and characterization methodologies . Since the discovery in 1992 of the hepatitis C virus (HCV) and its relation to cryoglobulins , numerous epidemiologic studies have been conducted on mixed cryoglobulins . Type I cryoglobulins have been investigated to a lesser degree due to their lower frequency and clinical association with malignant disorders .…”

Section: Introductionmentioning

confidence: 99%

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Cryoglobulins Today: Detection and Immunologic Characteristics of 1,675 Positive Samples From 13,439 Patients Obtained Over Six Years

Kolopp‐Sarda

Nombel

Miossec

2019

Arthritis & Rheumatology

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Objective Cryoglobulins are cold‐precipitating immunoglobulins. Through progress in techniques, we undertook this study to update information on the biologic characteristics of cryoglobulins in a very large population. Methods A cohort of 13,439 patients was tested for cryoglobulins from January 2010 to December 2016. The analysis included cryoglobulin isotype, clonality, concentration, and IgM rheumatoid factor (IgM‐RF) in cryoprecipitate, as well as serum complement and RF. Markers of gammopathy, viral infection, and autoimmunity were also investigated. Results Of the 13,439 patients, 1,675 (12.5%) tested positive for cryoglobulins: 155 patients (9.3%) with type I, 788 (47%) with type II, and 732 (43.7%) with type III cryoglobulins. Nine percent of patients who were retested after initially testing negative for cryoglobulins showed a positive result on a follow‐up test (196 of the 2,213 retested patients). In type I cryoglobulins, IgM was more frequent but occurred at lower concentrations than IgG. Mixed cryoglobulins were found in 34.8% of the tested patients who were positive for hepatitis C virus and <5% of those who were positive for hepatitis B virus or HIV. Of the patients with anti–double‐stranded DNA, anti‐SSA, or anti–cyclic citrullinated peptide autoantibodies, 25.4% tested positive for mixed cryoglobulins, with type III occurring more frequently than type II. Both cryoprecipitate and serum were RF‐positive in 21.6% of type II and 10.1% of type III cryoglobulins. A decrease of C4, with or without accompanying decreases of C3 and CH50, was found in 23.6% of cryoglobulin samples. Conclusion Obtained with the use of modern assays, our findings from this very large collection of cryoglobulins provide an update on cryoglobulin distribution and characteristics, with minimal selection bias. Despite strict preanalytical conditions, a negative finding for the presence of cryoglobulin must be confirmed in a second sample. RF activity and complement decreases were rarely detected.

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Section: Discussionsupporting

confidence: 91%

Section: Introductionmentioning

confidence: 99%

Cryoglobulins Today: Detection and Immunologic Characteristics of 1,675 Positive Samples From 13,439 Patients Obtained Over Six Years

Kolopp‐Sarda

Nombel

Miossec

2019

Arthritis & Rheumatology

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“…Mixed cryoglobulinemia (MC) is a systemic vasculitis characterized by multiple organ involvement due to the vascular deposition of immune-complexes, mainly mixed IgG-IgM cryoglobulins and complement [1][2][3] . These cryoprecipitable immune-complexes are the result of B-cell clone proliferation, triggered by chronic hepatitis C virus (HCV) infection in most cases [4] .…”

Section: Introductionmentioning

confidence: 99%

Improved (4 Plus 2) Rituximab Protocol for Severe Cases of Mixed Cryoglobulinemia: A 6-Year Observational Study

Roccatello

Sciascia²,

Baldovino³

et al. 2016

Am J Nephrol

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Background: In a prospective, single-center open study, we evaluated the very long-term effects of rituximab (RTX) administered to patients with severe mixed cryoglobulinemia (MC). Methods: RTX was administered to 31 patients with MC (type II in 29 cases and type III in 2) with diffuse membranoproliferative glomerulonephritis (16 cases), peripheral neuropathy (26) and large skin ulcers (7). All but 4 patients had serum anti-hepatitis C virus antibodies. RTX was administered at a dose of 375 mg/m², according to a ‘4 + 2' protocol (days 1, 8, 15 and 22 plus 1 dose 1 and 2 months later). No other immunosuppressive drugs were added. Response was evaluated over a very long-term follow-up (mean 72.47 months, range 30-148). Results: Complete remission of pretreatment active manifestations was observed in all cases of purpuric lesions and non-healing vasculitic ulcers, and in 80% of the peripheral neuropathies. Cryoglobulinemic nephropathy significantly improved during follow-up, starting from the 2nd month after RTX (serum creatinine from 2.1 ± 1.7 to 1.5 ± 1.6 mg/dl, p ≤ 0.05; 24-hour proteinuria from 2.3 ± 2.1 to 0.9 ± 1.9 g/24 h, p ≤ 0.05). Improvement of cryoglobulinemic serological hallmarks, such as cryocrit and low complement C4, were observed. No clinically relevant side effects were recorded. Re-induction with RTX was carried out in 9 relapsed patients after a mean of 31.1 months (12-54), again with beneficial effects. The survival rate was 75% at 6 years and the probability of remaining symptom-free for 10 years without any therapy was of about 60% after a single ‘4 + 2' infusion cycle, while the probability of living symptom-free 5 years after relapsing was 80% if given the same treatment. Conclusion: In this open, prospective study, RTX appeared to be very effective and safe in the treatment of the most severe cases of MC.

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“…Во всем мире КГВ считается редким заболеванием (<5 случаев на 10 тыс. человек в общей европейской и североамериканской популяции), хотя распространенность его несколько выше в странах Средиземноморского бассейна [8].…”

Section: эпидемиология и этиологияunclassified

Cryoglobulinemia and cryoglobulinemic vasculitis: etiological aspects and pathophysiological associations

Головач¹,

Егудина²

2020

Sovremennaâ revmatologiâ

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Термин «криоглобулинемия» (КГ) употребляется при обнаружении в сыворотке крови иммуноглобулинов, которые обратимо осаждаются и образуют гель при температуре ниже 37 °С и растворяются при повышении температуры выше 37 °С. КГ I типа представлена только одним изотипом или подклассом моноклональных иммуноглобулинов, тогда как II и III типы классифицируются как смешанная КГ (СКГ), для которой преимущественно характерно наличие иммуноглобулинов G и M. Чаще к криоглобулинемическому васкулиту (КГВ) может привести СКГ II и II-III типов, реже -III типа. Присутствие криоглобулинов I типа всегда ассоциировано с В-клеточными лимфопролиферативными заболеваниями. СКГ II или III типа, напротив, чаще ассоциирована с системными аутоиммунными заболеваниями, хроническими инфекциями. Так, вирусный гепатит С способствует развитию СКГ в 80-90% случаев. Во всем мире КГВ считается редким заболеванием (<5 случаев на 10 тыс. человек в общей европейской и североамериканской популяции). Среди аутоиммунных заболеваний чаще всего с СКГ ассоциированы первичный синдром Шёгрена (болезнь Шёгрена), системная красная волчанка и ревматоидный артрит. Патогенетическая роль криоглобулинов в индуцировании васкулита связана как с рекрутированием лейкоцитов в сосуды, так и с отложением иммунных комплексов, с активацией системы комплемента и повреждением микрососудов. Патогенез КГВ связан с В-клеточной лимфопролиферацией, продукцией аутоантител, синтезом иммуноглобулинов, с активностью ревматоидного фактора и последующим образованием криопреципитируемых иммунных комплексов в сочетании с неэффективным клиренсом криоглобулинов моноцитами и/или макрофагами. В данном обзоре собрана обновленная информация об эпидемиологии, этиологии и патогенезе КГ с особым акцентом на СКГ и КГВ. Ключевые слова: криоглобулинемия; васкулит; иммуноглобулины; вирус гепатита С; патогенез; лимфопролиферативный процесс; В-клетки. Контакты: Ирина Юрьевна Головач; golovachirina@gmail.com Для ссылки: Головач ИЮ, Егудина ЕД. Криоглобулинемия и криоглобулинемический васкулит: этиологические аспекты и патофизиологические ассоциации. Современная ревматология. 2020;14(1):78-84 .

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Prevalence of mixed cryoglobulinaemia syndrome and circulating cryoglobulins in a population-based survey: the Origgio study

Cited by 39 publications

References 47 publications

Cryoglobulins Today: Detection and Immunologic Characteristics of 1,675 Positive Samples From 13,439 Patients Obtained Over Six Years

Cryoglobulins Today: Detection and Immunologic Characteristics of 1,675 Positive Samples From 13,439 Patients Obtained Over Six Years

Improved (4 Plus 2) Rituximab Protocol for Severe Cases of Mixed Cryoglobulinemia: A 6-Year Observational Study

Cryoglobulinemia and cryoglobulinemic vasculitis: etiological aspects and pathophysiological associations

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