Background Information on certain immunological parameters in patients with pemphigus vulgaris (PV) treated with rituximab (RTX) and intravenous immunoglobulin (IVIG) therapy is limited.
Objective Comparing immunological parameters in patients who achieved long-term clinical remission (LTR) with those who relapsed.
Methods Retrospective analysis of 19 patients treated at a single centre using the same protocol. Comparisons were made between patients who went into LTR and those who relapsed following completion of the protocol. Treatments prior to IVIG and RTX included prednisone with or without an immunosuppressive agent. The immunological parameters measured included peripheral blood B cells (CD19+), serum quantitative immunoglobulin levels, and levels of antibodies to desmogleins (Dsg) 1 and 3.
Results Eleven patients achieved LTR. Eight patients developed 15 relapses. The mean follow-up time for the LTR group was 29·6 ± 11·2 months, and for the relapse group, 40·0 ± 7·0 months. There were no significant differences in times to B-cell depletion, repopulation, or recovery to pretreatment levels between the patients who achieved LTR and those who relapsed. Recurrences usually occurred after B-cell repopulation. Repeated treatments did not influence the time to B-cell repopulation. IgM levels were decreased after therapy and remained decreased. A consistent increase in anti-Dsg1 antibody levels occurred at the time of relapse in patients with mucocutaneous disease.
Conclusions The majority of patients treated with rituximab and IVIG therapy achieved LTR. Retreatment of relapses can induce LTR. Decreased serum IgM levels persisted following treatment. Increases in anti-Dsg1 antibodies during therapy in patients with mucocutaneous disease suggests a close follow-up for a potential relapse is required.