Background: Mixed cryoglobulinemia syndrome (MCs) is a systemic vasculitis of small and medium-sized vessels. Its prevalence presents a large geographic heterogeneity; in particular, MC is more frequent in Southern Europe than in Northern Europe or in Northern America. Because of the striking association between hepatitis C virus (HCV) infection and MCs (up to 95-98% of cases in Italian series), the term 'essential' should be reserved only to the small number of patients without overt infectious, immuno-rheumatological or neoplastic disorders.Several strategies have been adopted to contrast HCV infection, such as the screening of hemoderivatives and the development of antiviral therapies.Objectives: we investigated the possible variations during the last years of the prevalence of HCV infection in Italian patients with overt MCs.Methods: From 2000 to 2008, MCs was diagnosed for the first time in 75 consecutive patients referred to our Rheumatology Unit (55F and 20M; mean age 65.7±11.3 SD years). At the beginning of the follow-up, all patients showed clinico-serological and pathological features sufficient for the a definite diagnosis of MCs according to the preliminary classification criteria.They were characterized by the typical clinical triad -purpura, weakness, arthralgias- in 80% of patients, mild-moderate chronic hepatitis in 52%, peripheral neuropathy in 65%, nephropathy in 9%, and severe cutaneous vasculitis in 21%.Results: in the present series of recently diagnosed patients, the percentage of HCV-positive individuals (81.3%; genotype 1b 55%, 2a/2c 38%; other 7%) was lower compared to previously observed prevalence of HCV-positives (92%) in our 168 patients screened for HCV infection. We can speculate that this reduction could be the result of several actions adopted in order to reduce the incidence of HCV infection in the general population. Furthermore, the use of combined antiviral treatment, particularly if introduced in the early stages of infection, increases the percentage of HCV eradication and, consequently, it reduces the risk of developing MCs. On the other hand, the populations with a higher prevalence of HCV infection (i.e. Africans or Asians) more often are infected with viral genotypes that seem to be scarcely involved in the pathogenesis of MCs.Another important finding of the present series is the patients' age at the diagnosis compared to that observed in our previous study (65.7±11.3 vs 56±11 SD years; p<0.0001). It could be an indirect consequence of the preventive measures adopted after HCV discovery in 1989, which lowered the incidence of viral infection in younger individuals and consequently the possible development of MCs.Conclusion: on the bases of the above considerations, we can suppose that in next years the overall incidence of the disease will show a valuable reduction; contemporarily, the relative percentage of 'essential' MCs would gradually increase. This might be particularly true for patients' populations from Italy and other Mediterranean countries, where the pathogenetic link with HCV infection is prevalent.

Mixed cryoglobulinemia syndrome: where are we going to? / Sebastiani, Marco; Colaci, Michele; Manfredi, Andreina Teresa; Elkhaldi, Nezar Moneir Fadl; Mascia, Maria Teresa; Ferri, Clodoveo. - In: ANNALS OF THE RHEUMATIC DISEASES. - ISSN 0003-4967. - STAMPA. - 68:(2009), pp. 602-602. (Intervento presentato al convegno EULAR Congress tenutosi a Copenaghen nel 10-13/06/2009).

Mixed cryoglobulinemia syndrome: where are we going to?

SEBASTIANI, Marco;COLACI, Michele;MANFREDI, Andreina Teresa;ELKHALDI, Nezar Moneir Fadl;MASCIA, Maria Teresa;FERRI, Clodoveo
2009

Abstract

Background: Mixed cryoglobulinemia syndrome (MCs) is a systemic vasculitis of small and medium-sized vessels. Its prevalence presents a large geographic heterogeneity; in particular, MC is more frequent in Southern Europe than in Northern Europe or in Northern America. Because of the striking association between hepatitis C virus (HCV) infection and MCs (up to 95-98% of cases in Italian series), the term 'essential' should be reserved only to the small number of patients without overt infectious, immuno-rheumatological or neoplastic disorders.Several strategies have been adopted to contrast HCV infection, such as the screening of hemoderivatives and the development of antiviral therapies.Objectives: we investigated the possible variations during the last years of the prevalence of HCV infection in Italian patients with overt MCs.Methods: From 2000 to 2008, MCs was diagnosed for the first time in 75 consecutive patients referred to our Rheumatology Unit (55F and 20M; mean age 65.7±11.3 SD years). At the beginning of the follow-up, all patients showed clinico-serological and pathological features sufficient for the a definite diagnosis of MCs according to the preliminary classification criteria.They were characterized by the typical clinical triad -purpura, weakness, arthralgias- in 80% of patients, mild-moderate chronic hepatitis in 52%, peripheral neuropathy in 65%, nephropathy in 9%, and severe cutaneous vasculitis in 21%.Results: in the present series of recently diagnosed patients, the percentage of HCV-positive individuals (81.3%; genotype 1b 55%, 2a/2c 38%; other 7%) was lower compared to previously observed prevalence of HCV-positives (92%) in our 168 patients screened for HCV infection. We can speculate that this reduction could be the result of several actions adopted in order to reduce the incidence of HCV infection in the general population. Furthermore, the use of combined antiviral treatment, particularly if introduced in the early stages of infection, increases the percentage of HCV eradication and, consequently, it reduces the risk of developing MCs. On the other hand, the populations with a higher prevalence of HCV infection (i.e. Africans or Asians) more often are infected with viral genotypes that seem to be scarcely involved in the pathogenesis of MCs.Another important finding of the present series is the patients' age at the diagnosis compared to that observed in our previous study (65.7±11.3 vs 56±11 SD years; p<0.0001). It could be an indirect consequence of the preventive measures adopted after HCV discovery in 1989, which lowered the incidence of viral infection in younger individuals and consequently the possible development of MCs.Conclusion: on the bases of the above considerations, we can suppose that in next years the overall incidence of the disease will show a valuable reduction; contemporarily, the relative percentage of 'essential' MCs would gradually increase. This might be particularly true for patients' populations from Italy and other Mediterranean countries, where the pathogenetic link with HCV infection is prevalent.
2009
68
602
602
Sebastiani, Marco; Colaci, Michele; Manfredi, Andreina Teresa; Elkhaldi, Nezar Moneir Fadl; Mascia, Maria Teresa; Ferri, Clodoveo
Mixed cryoglobulinemia syndrome: where are we going to? / Sebastiani, Marco; Colaci, Michele; Manfredi, Andreina Teresa; Elkhaldi, Nezar Moneir Fadl; Mascia, Maria Teresa; Ferri, Clodoveo. - In: ANNALS OF THE RHEUMATIC DISEASES. - ISSN 0003-4967. - STAMPA. - 68:(2009), pp. 602-602. (Intervento presentato al convegno EULAR Congress tenutosi a Copenaghen nel 10-13/06/2009).
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