Long-term outcomes and prognosis of mixed cryoglobulinemia: a European multicenter study of 1,294 patients

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.

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Lara D. Veeken
Published
2026-06-18
DOI
https://doi.org/10.1093/rheumatology/keag264
Primary Topic
Vasculitis and related conditions
Type
article
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article

Long-term outcomes and prognosis of mixed cryoglobulinemia: a European multicenter study of 1,294 patients

Caterina Vacchi, Gaëtane Nocturne, Abora Rial‐Villavecchia, Milvia Casato et al.
Lara D. Veeken
Vasculitis and related conditions
article

Long-term outcomes and prognosis of mixed cryoglobulinemia: a European multicenter study of 1,294 patients

Caterina Vacchi, Gaëtane Nocturne, Abora Rial‐Villavecchia, Milvia Casato, Alejandra Flores-Chávez, Agata Sebastian, Francesco Carubbi, Gener Ismail, Sylvie Chevret, Antónia Szántó, Soledad Retamozo, P Cacoub, Pilar Brito‐Zerón, Alessandra Bartoli, Sonja Praprotnik, Andrea Núñez Conde, Marco Sebastiani, Ricardo Gómez de la Torre, R Mataix, Pascale Ghillani‐Dalbin, Matheus Vieira, Manuel Ramos-Casals, Georgina Maalouf, Marcella Visentini, Ciprian Jurcuț, Luca Quartuccio, Fabiola Atzeni, Joan Maria Mercadé-Torras, Elena Bartoloni, Xavier Mariette, Laura León, David Saadoun
article en

Abstract

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.

Lara D. Veeken
University of Messina (IT), Universidade Federal do Rio de Janeiro (BR), University of Modena and Reggio Emilia (IT), Universitat Autònoma de Barcelona (ES), University of Parma (IT), University of Debrecen (HU), Inserm (FR), Délégation Paris 7 (FR), Université Paris Cité (FR), University of Perugia (IT), Université Paris-Saclay (FR), Ljubljana University Medical Centre (SI), Wroclaw Medical University (PL), Sorbonne Université (FR), Sociedad Española de Medicina Interna (ES), Azienda Unita' Sanitaria Locale Di Modena (IT), Assistance Publique – Hôpitaux de Paris (FR), San Salvatore Hospital (IT), Centre de Référence des Maladies Autoinflammatoires et des Amyloses (FR), Emergency University (US), Autoimmune Technologies (United States) (US), Pitié-Salpêtrière Hospital (FR), Ospedale Santa Maria della Misericordia di Udine (IT), Hospital Del Mar (ES), Hospital Universitario Central de Asturias (ES), Institute of Research and Innovation Parc Tauli (ES), Hôpital Saint-Louis (FR), Complejo Hospitalario Torrecárdenas (ES), Hospital Universitario Virgen del Rocío (ES), Consorci Institut D'Investigacions Biomediques August Pi I Sunyer (ES), Institutul Clinic Fundeni (RO), Bicêtre Hospital (FR), Universidade Federal do Estado do Rio de Janeiro (BR), Sapienza University of Rome (IT)
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Vasculitis and related conditions
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