TY - JOUR T1 - Rituximab-associated Vasculitis Flare: Incidence, Predictors, and Outcome JF - The Journal of Rheumatology JO - J Rheumatol SP - 896 LP - 902 DO - 10.3899/jrheum.190076 VL - 47 IS - 6 AU - Anne Claire Desbois AU - Lucie Biard AU - Damien Sène AU - Isabelle Brocheriou AU - Philippe Rouvier AU - Bertrand Lioger AU - Lucile Musset AU - Sophie Candon AU - Thierry Zenone AU - Matthieu Resche-Rigon AU - Jean-Charles Piette AU - Neila Benameur AU - Patrice Cacoub AU - David Saadoun Y1 - 2020/06/01 UR - http://www.jrheum.org/content/47/6/896.abstract N2 - Objective. To report the incidence, predictors, and outcome of rituximab (RTX)-associated autoimmune disease flare.Methods. We conducted a retrospective study in a tertiary referral center from 2005 to 2015. Disease flare was defined as the onset of a new organ involvement or worsening of autoimmune disease within 4 weeks following RTX.Results. Among the 185 patients, we identified 7 disease flares (3.4%). All were due to type II mixed cryoglobulinemia vasculitis. Vasculitis flare occurred after a median time of 8 days (range 2–16) following RTX infusion and included acute kidney insufficiency (n = 7), purpura with cutaneous (n = 7), gastrointestinal (GI) tract involvement (n = 4), and myocarditis (n = 1). Patients with RTX-associated cryoglobulinemia vasculitis flare had these conditions more frequently: renal involvement (p = 0.0008), B cell lymphoproliferation (p = 0.015), higher level of cryoglobulin (2.1 vs 0.4 g/l, p = 0.0004), and lower level of C4 (0.02 vs 0.05, p = 0.023) compared to patients without flare after RTX (n = 43). Four patients (57%) died after a median time of 3.3 months. The 1-year survival rate was poorer in patients with vasculitis flare after RTX compared to their negative counterpart [43% (95% CI 18–100) vs 97% (95% CI 92–100), p < 0.001]. Immunofluorescence analysis of kidney biopsy in patients with worsening RTX-associated vasculitis highlighted the presence of RTX-, IgM-, and IgG1-positive staining of endomembranous deposits and thrombi within kidney lesions.Conclusion. RTX-associated cryoglobulinemia vasculitis flare is associated with high mortality rate. We provided evidence that kidney lesions are due to immune complex deposition and to glomerular obstruction by cryoglobulinemia and RTX. ER -