Which drugs are used in the treatment of EGPA (Churg-Strauss syndrome)?

Updated: Dec 24, 2018
  • Author: Spencer T Lowe, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

Glucocorticoids alone are usually adequate for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome). [36, 37] Cytotoxic drugs are necessary in fewer than 20% of patients.

Rituximab, which is approved for use in granulomatosis with polyangiitis and microscopic polyangiitis, has proved useful in treatment of steroid-resistant cases, as well as for prevention and treatment of relapse. [38] The anti-IgE monoclonal antibody omalizumab has demonstrated a corticosteroid-sparing effect in refractory or relapsing EGPA, but reducing steroid doses may also increase the risk of severe EGPA flares. [39] Case reports have described infliximab therapy in patients with steroid-dependent EGPA. [40]

Major life-threatening organ involvement may require treatment with pulse doses of intravenous corticosteroids and other cytotoxic agents. Cyclophosphamide is typically given in intravenous pulses for 3 months; afterward, patients are converted to oral mycophenolate, azathioprine, or methotrexate for maintenance therapy.

Plasma exchange has been studied in EGPA and other ANCA-positive vasculitides, without a clear benefit. [41] A meta-analysis of 140 patients with glomerulonephritis and EGPA and microscopic polyangiitis confirmed that plasma exchanges offered no added benefit. [42] One case report is available on intravenous immune globulin for treatment of EGPA in pregnancy. [43]

Mepolizumab is an interleukin-5 antagonist monoclonal antibody (IgG1 kappa) produced by recombinant DNA technology in Chinese hamster ovary cells. It was previously approved in 2015 as an add-on maintenance treatment in patients with severe asthma that has an eosinophilic phenotype. Approval was based on a 52-week multicenter, double-blind, parallel-group, phase III trial in patients with relapsing or refractory EGPA who had received glucocorticoid treatment for at least 4 weeks. Patients were randomized to either receive mepolizumab or placebo once every 4 weeks while continuing corticosteroid (OCS) therapy.

More patients in the mepolizumab group than the placebo group remained in remission for 44</ref>


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