Medizinische Universität Graz - Research portal
Selected Publication:
SHR Neuro Cancer Cardio Lipid Metab Microb
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; 15(5): R133
Doi: 10.1186/ar4313
[OPEN ACCESS]
Web of Science
PubMed
FullText
FullText_MUG
- Leading authors Med Uni Graz
- Thiel Jens
- Altmetrics:
- Dimensions Citations:
- Plum Analytics:
- Scite (citation analytics):
- Abstract:
- INTRODUCTION: Eosinophilic granulomatosis with polyangiitis (EGPA) is part of antineutrophil cytoplasmic antibodies (ANCAs)-associated vasculitides. In EGPA small-vessel vasculitis is associated with eosinophilia and asthma. About 40% of EGPA patients are ANCA-positive, suggesting a role for B cells in the pathogenesis of EGPA. B cell-depleting therapy with rituximab (RTX) can be effective in ANCA-positive EGPA, but very few patients have been published to date. The role of RTX in the treatment of ANCA-negative EGPA is unclear. METHODS: We report a single-center cohort of patients with eosinophilic granulomatosis with polyangiitis. Of these patients, nine (six ANCA-positive, three ANCA-negative) had been treated with RTX for relapsing or refractory disease on standard immunosuppressive treatment. In a retrospective analysis, data on treatment response, frequency of relapses, adverse events, and peripheral B-cell reconstitution were evaluated. Furthermore, serum immunoglobulin concentrations, ANCA status, and peripheral B cell subpopulations were assessed after RTX treatment. RESULTS: All patients had high disease activity before RTX treatment. At presentation 3 months after RTX therapy, all ANCA-positive and ANCA-negative patients had responded to RTX, with one patient being in complete remission, and eight patients being in partial remission. After a mean follow-up of 9 months, C-reactive protein concentrations had normalized, eosinophils had significantly decreased, and prednisone had been tapered in all patients. In all patients, RTX therapy was combined with a standard immunosuppressive therapy. Within the 9-month observation period, no relapse was recorded. Three patients were preemptively retreated with RTX, and during the median follow-up time of 3 years, no relapse occurred in these patients. During the follow-up of 13 patient-years, five minor but no major infections were recorded. CONCLUSIONS: In our analysis on nine patients with EGPA resistant to standard therapy, rituximab proved to be an efficient and safe treatment for ANCA-positive and ANCA-negative patients. Preemptive retreatment with RTX, combined with standard maintenance immunosuppressants, resulted in a sustained treatment response. Prospective, randomized trials evaluating the use of RTX in EGPA are warranted.
- Find related publications in this database (using NLM MeSH Indexing)
- Adult - administration & dosage
- Antibodies, Antineutrophil Cytoplasmic - blood
- Antibodies, Monoclonal, Murine-Derived - therapeutic use
- Antirheumatic Agents - therapeutic use
- B-Lymphocyte Subsets - drug effects, metabolism
- C-Reactive Protein - metabolism
- Churg-Strauss Syndrome - blood, drug therapy, pathology
- Drug Resistance - administration & dosage
- Female - administration & dosage
- Follow-Up Studies - administration & dosage
- Humans - administration & dosage
- Immunoglobulin E - blood
- Immunosuppressive Agents - therapeutic use
- Lymphocyte Count - administration & dosage
- Male - administration & dosage
- Middle Aged - administration & dosage
- Recurrence - administration & dosage
- Retrospective Studies - administration & dosage
- Rituximab - administration & dosage
- Time Factors - administration & dosage
- Treatment Outcome - administration & dosage