Objectives: To assess the long-term outcomes and prognostic factors of patients presenting with all-cause mixed cryoglobulinemia vasculitis (CryoVas). Methods: A European multicentre cohort study (EuroCryo) of 1,294 patients presenting with all-cause CryoVas. Results: The main aetiology for CryoVas was HCV with 469 (36%) cases, followed by essential forms (n = 344, 26%) and autoimmune conditions (n = 302, 23%) with Sjogren's disease being the most common (n = 268, 21%). Over a median follow-up of 2.8 years, a total of 558 CryoVas patients experienced 661 severe events, notably 221 relapses, 52 severe infections and 45 lymphomas. Multivariable model for event-free survival retained a post-2014 diagnosis, male sex, older age, low C4 levels, positive rheumatoid factor and higher baseline creatinine levels as poor prognostic factors; HCV aetiology was protective. Factors associated with the occurrence of lymphoma were a post-2014 diagnosis, autoimmune aetiology, fever, fatigue and low C4 levels. Conclusion: HCV was for long considered a poor prognosis factor in CryoVas, and now those secondary to autoimmune conditions seem to evolve with a more severe course, notably carrying a higher lymphoma risk.
Long-term outcomes and prognosis of mixed cryoglobulinaemia: a European multicentre study of 1294 patients / Saadoun, D., Cacoub, P., Retamozo, S., Sebastiani, M., Vacchi, C., Casato, M., Visentini, M., Brito-Zerón, P., Flores-Chávez, A., Praprotnik, S., Ismail, G., Quartuccio, L., Nocturne, G., Szanto, A., Maalouf, G., Bartoli, A., Ghillani-Dalbin, P., Torre, R.G.D.L., Pertusa Mataix, R., Conde, A.N., et al.. - In: RHEUMATOLOGY. - ISSN 1462-0324. - 65:6(2026). [10.1093/rheumatology/keag264]
Long-term outcomes and prognosis of mixed cryoglobulinaemia: a European multicentre study of 1294 patients
Sebastiani, Marco;
2026-01-01
Abstract
Objectives: To assess the long-term outcomes and prognostic factors of patients presenting with all-cause mixed cryoglobulinemia vasculitis (CryoVas). Methods: A European multicentre cohort study (EuroCryo) of 1,294 patients presenting with all-cause CryoVas. Results: The main aetiology for CryoVas was HCV with 469 (36%) cases, followed by essential forms (n = 344, 26%) and autoimmune conditions (n = 302, 23%) with Sjogren's disease being the most common (n = 268, 21%). Over a median follow-up of 2.8 years, a total of 558 CryoVas patients experienced 661 severe events, notably 221 relapses, 52 severe infections and 45 lymphomas. Multivariable model for event-free survival retained a post-2014 diagnosis, male sex, older age, low C4 levels, positive rheumatoid factor and higher baseline creatinine levels as poor prognostic factors; HCV aetiology was protective. Factors associated with the occurrence of lymphoma were a post-2014 diagnosis, autoimmune aetiology, fever, fatigue and low C4 levels. Conclusion: HCV was for long considered a poor prognosis factor in CryoVas, and now those secondary to autoimmune conditions seem to evolve with a more severe course, notably carrying a higher lymphoma risk.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
