Long-term outcomes and prognosis of mixed cryoglobulinaemia: a European multicentre study of 1294 patients
David Saadoun, Patrice Cacoub, Soledad Retamozo, Marco Sebastiani, Caterina Vacchi, Milvia Casato, Marcella Visentini, Pilar Brito-Zerón, Alejandra Flores-Chávez, Sonja Praprotnik, Gener Ismail, Luca Quartuccio, Gaetane Nocturne, Antonia Szanto, Georgina Maalouf, Alessandra Bartoli, Pascale Ghillani-Dalbin, Ricardo Gómez de la Torre, Roberto Pertusa Mataix, Andrea Núñez Conde, Elena Bartoloni, Laura León, Joan Maria Mercadé-Torras, Agata Sebastian, Francesco Carubbi, Fabiola Atzeni, Aborá Rial-Villavecchia, Xavier Mariette, Ciprian Jurcut, Matheus Vieira, Sylvie Chevret, Manuel Ramos-CasalsAbstract
Objectives
To assess the long-term outcomes and prognostic factors of patients presenting with all-cause mixed cryoglobulinaemia vasculitis (CryoVas).
Methods
A European multicentre cohort study (EuroCryo) of 1294 patients presenting with all-cause CryoVas.
Results
The main aetiology for CryoVas was Hepatitis C Virus ( HCV) with 469 (36%) cases, followed by essential forms (n = 344, 26%) and autoimmune conditions (n = 302, 23%), with Sjögren’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. The 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.