EULAR 2016 | Daily Highlights
COMPARISON OF RITUXIMAB WITH CYCLOPHOSPHAMIDE AS INDUCTION THERAPY IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG-STRAUSS SYNDROME): A 24 MONTHS FOLLOW-UP ANALYSIS.Abstract: FRI0355
Authors: N. Venhoff1,*, K. Halmschlag1, M. Rizzi1, R. Voll1, J. Thiel1
Co Authors: 1Department of Rheumatology and Clinical Immunology, University Medical Center Freiburg, Freiburg, Germany
Eosinophilic granulomatosis with polyangiitis (EGPA) forms part of the ANCA associated vasculitides (AAV) and is associated with eosinophilia and asthma. To date no consensus exists on induction and maintenance therapy and refractory patients still represent a considerable therapeutic challenge. We recently reported on the efficacy of rituximab (RTX) as induction therapy in EGPA. So far there are no data on RTX in comparison with cyclophosphamide (CYC) in this disease.
To investigate the potential of RTX compared with CYC in EGPA as induction therapy and during long-term follow-up.
Retrospective analysis in a single-center cohort of 46 EGPA patients. Remission and disease activity were determined by BVAS. Laboratory follow-up included C-reactive protein, eosinophils, IgE, IgG, IgA, IgM, ANCA, and peripheral CD19+ B-cell count.
Eleven patients treated with RTX for relapsing or refractory disease were compared with twelve age- and sex-matched patients treated with CYC for remission induction. Median disease duration was longer in RTX patients (49 months, IQR 7-66 vs. 8.5 months, IQR 0-7; p=0.0025). Prior to induction therapy there was no significant difference in median BVAS (14 vs. 12.5) and in FFS (1 vs. 0.75). Median eosinophil percentages were increased in both RTX (18.4%) and CYC (6.75%) treatment groups (n.s.). 73% of the RTX-treated patients were ANCA-positive compared to only 23% of the CYC-treated patients. Median prednisolone dose per day at induction was significantly higher in CYC-treated patients (35 mg/day, IQR 26-138) than in RTX-treated patients (17.5 mg/day, IQR 10-30). After 3 months all patients in both treatment groups were in partial or complete remission. For maintenance therapy all but two patients per group received standard immunosuppressive treatment with a predominance of azathioprine (AZA) (n=7 per group). Median cumulative prednisolone doses during the first treatment year including high doses for remission induction were 3.6 g in RTX- and 3.8 g in CYC-treated patients (p=0.725). Within an observation period of 24 months after remission induction 3 patients in the RTX group and 2 patients in the CYC group had major relapses (n.s.). In two of the 3 relapsing patients after RTX treatment peripheral B-cell counts were evaluated showing a beginning repopulation of the peripheral B-cell compartment. Serum concentrations of IgG, IgA, and IgM remained stable in both treatment groups during follow-up.
RTX was effective in remission induction in EGPA patients. Remarkably this was the fact even in patients refractory to prior CYC treatment and in ANCA negative patients. During follow up the number of major relapses was comparable in both treatment groups. Prior to relapse the peripheral B cell compartment showed beginning repopulation in RTX-treated patients.
Thiel J, Hässler F, Salzer U, Voll RE, Venhoff N. Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Arthritis Res Ther. 2013 Sep 24;15(5):R133.
Disclosure of Interest
Due to its very low prevalence, data about treatment of eGPA are limited. Often treatment regimens are deduced from GPA data. In this retrospective study the authors compared induction and maintenance therapy with rituximab (RTX) versus cyclophosphamide (CYC). They analyzed data of 11 patients treated with RTX and 12 age- and sex-matched patients treated with CYC.
Several points merit mentioning: RTX was used in relapsing and refractory, but not in newly diagnosed cases; ANCA was positive in 73% of RTX but only 23% of CYC cases; eosinophils were increased in 18,4 (RTX) versus 6.75% (CYC) of patients. Thus, disease characteristics were rather different, a fact which makes firm conclusions difficult. Regarding maintenance, therapy consisted of conventional PDN plus azathioprine and relapse rate was comparable (3 versus 2).
In conclusion, the data show that B cell deleting therapy using RTX is effective in relapsing and refractory eGPA. They suggest effectiveness in ANCA + as well as ANCA - patients. There remains a need for a prospective protocol addressing not only induction using RTX but also maintenance therapy using RTX, as recently published for GPA and MPA by the group of Guillevin and studied by the EUVAS.
Prof. Dr. Peter Villiger
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