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Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

AbstractINTRODUCTION:
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.
AuthorsJens Thiel, Fabian Hässler, Ulrich Salzer, Reinhard E Voll, Nils Venhoff
JournalArthritis research & therapy (Arthritis Res Ther) Vol. 15 Issue 5 Pg. R133 (Sep 24 2013) ISSN: 1478-6362 [Electronic] England
PMID24286362 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Antibodies, Antineutrophil Cytoplasmic
  • Antibodies, Monoclonal, Murine-Derived
  • Antirheumatic Agents
  • Immunosuppressive Agents
  • Immunoglobulin E
  • Rituximab
  • C-Reactive Protein
Topics
  • Adult
  • 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
  • Female
  • Follow-Up Studies
  • Humans
  • Immunoglobulin E (blood)
  • Immunosuppressive Agents (therapeutic use)
  • Lymphocyte Count
  • Male
  • Middle Aged
  • Recurrence
  • Retrospective Studies
  • Rituximab
  • Time Factors
  • Treatment Outcome

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