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Rituximab Efficacy in IgG4 Related Disease

PLOS reports  greater than 90% efficacy when rituximab (RTX) is used to treat patients with IgG4-related disease (IgG4-RD). 

The diagnosis and management of IgG4-RD is challenging and often without proven trial-based evidence.  A French national retrospective multicenter study examined the efficacy of RTX given to 33 patients with IgG4-RD. This RTX treated cohort was drawn from a total of 156 IgG4-RD patients in the French database.

A clinical response was noted in nearly all patients - 29/31 (93.5%) improved and steroid withdrawal was achieved in over half the patients (51.5%).

During a mean follow-up of 24 months, 13/31 (41.9%) of the RTX responding patients relapsed a mean of 19 months after RTX. 

Patients with active disease (IgG4-RD Responder Index >9) before RTX were more likely to relapse (HR = 3.68, 95% CI: 1.1, 12.6) (P = 0.04), whereas maintenance RTX therapy was associated with longer relapse-free survival (41 versus 21 months; P = 0.02). 

Important adverse effects included 8 serious infections in 4 patients (rate = 12.1/100 patient-years) and hypogammaglobulinemia was seen in 3 patients.

RTX is effective for both induction and maintenance therapy for IgG4-RD, but relapses are frequent after B-cell reconstitution. 

The author has no conflicts of interest to disclose related to this subject

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