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. 2025 Nov 1;64(11):5665-5672.
doi: 10.1093/rheumatology/keaf324.

Prognosis of essential mixed cryoglobulinemia and connective tissue disease-related cryoglobulinemia after rituximab-induced remission

Claire Poggi  1   2 , Eric Hachulla  1 , Alexandre Karras  3 , Antoine Briantais  4 , Camille Ravaiau  5 , Pierre Gobert  6 , Alban Deroux  7 , Sarah Nicolas  8 , Nabil Belfeki  9 , Hélène François  10 , Matthieu Groh  11 , Jonathan London  12 , Julien Campagne  13 , Jean-Sébastien Allain  14 , Emmanuelle Dernis  15 , Cécile-Audrey Durel  16 , Thomas Le Gallou  17 , Alexandre Curie  18 , Philippe Kerschen  19 , Noémie Gensous  20 , Anne-Hélène Reboux  21 , Hélène Béhal  22 , Benjamin Terrier  23   24 , Thomas Quéméneur  2
Affiliations

Prognosis of essential mixed cryoglobulinemia and connective tissue disease-related cryoglobulinemia after rituximab-induced remission

Claire Poggi et al. Rheumatology (Oxford). .

Abstract

Objectives: Rituximab (RTX) and glucocorticoids are the first-line treatment for essential (EM) and connective tissue disease (CTD)-related mixed cryoglobulinemia vasculitis (CryoVas). Data on long-term outcomes of these CryoVas are lacking. We aimed to describe the prognosis of patients with EM and CTD-related CryoVas.

Methods: We conducted a retrospective study on patients with EM or CTD-related CryoVas in remission after RTX-based therapy.

Results: We included 63 patients with a median follow-up of 58 months (IQR, 33-88 months). Relapse rates were 23% at 1 year, 42% at 2 years and 71% at 5 years after the initial flare. In univariate analysis, factors associated with relapse were purpura [HR, 2.2; 95% confidence interval (CI), 1.1-4.4; P = 0.02] and a previous flare of CryoVas (HR, 1.9; 95% CI, 1.0-3.7; P = 0.04). Maintenance therapy was associated with a lower risk of early relapse (HR, 0.3; 95% CI, 0.1-0.9; P = 0.03), but not of late relapse (HR, 2.0; 95% CI, 0.7-5.7; P = 0.21). In multivariable analysis, patients without purpura or previous flare remained at lower risk of relapse than those with at least one of the two (HR, 3.6; 95%CI, 1.6-8.2; P = 0.002). Maintenance regimen was associated with a lower risk of early relapse (HR, 0.3; 95% CI, 0.1-0.9; P = 0.03).

Conclusion: In patients with EM and CTD-related CryoVas who received RTX as induction therapy, relapses were frequent and associated with purpura and a previous flare but were reduced with maintenance therapy.

Keywords: autoimmune disease; cryoglobulinemia; relapse; remission maintenance; rituximab; vasculitis.

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