Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Treatment & Management

Updated: Aug 06, 2022
  • Author: Spencer T Lowe, MD; Chief Editor: Herbert S Diamond, MD  more...
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Approach Considerations

Glucocorticoids alone are usually adequate for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome). [40, 41] 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. [42]  However, its efficacy is greatly reduced in ANCA-negative patients with EGPA. [43, 12]

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. [44] Case reports have described infliximab therapy in patients with steroid-dependent EGPA. [45]

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. [46] A meta-analysis of 140 patients with glomerulonephritis and EGPA and microscopic polyangiitis confirmed that plasma exchanges offered no added benefit. [47] One case report is available on intravenous immune globulin for treatment of EGPA in pregnancy. [48]

Mepolizumab was approved for use in adult patients with EGPA by the US Food and Drug Administration in 2017. Mepolizumab is an interleukin-5 (IL-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 for EGPA 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 therapy.

More patients in the mepolizumab group than the placebo group remained in remission for < 24 weeks (28% versus 3%, respectively). Failure to achieve remission occurred in 47% of patients in the mepolizumab group, as compared with 81% of patients in the placebo group. A higher percentage of participants in the mepolizumab group than in the placebo group had remission at both week 36 and week 49 (32% vs. 3%). Moreover, significantly more patients who received mepolizumab achieved remission within the first 24 weeks and remained in remission for the remainder of the 52-week study. [49]

Benralizumab, an IL-5α antagonist monoclonal antibody, is approved for use in severe asthma and has orphan drug designation for EGPA. A study by Koga et al in 41 patients with mepolizumab-refractory EGPA and intractable cardiac and neuropathy complications reported that after the initiation of benralizumab therapy, oral corticosteroids could be reduced to 10 mg/day or less in all patients and to less than 5 mg/day at least 80%. More than 40% of patients were able to discontinue corticosteroids. [50]


Medical Care

Inpatient medications include the following:

  • Prednisone: Start at 0.5-1 mg/kg/d PO.

  • Methylprednisolone is administered in intravenous (IV) form at higher doses in patients with major organ involvement, including cardiac, pulmonary (hemorrhage), renal, or neuropathy.

  • Cyclophosphamide is administered in patients with severe or life-threatening complications. [51, 52]  The single IV dose is 500 mg/m2 of body-surface area. The dose should be reduced in patients with renal failure.

  • Other medications include azathioprine, mycophenolate mofetil, and intravenous immune globulin administered similarly as in microscopic polyangiitis or granulomatosis with polyangiitis (Wegener granulomatosis). [53]

  • Plasmapheresis is probably not helpful. [47]

  • Interferon alpha might be helpful in treatment. [54, 55]



EGPA is a systemic vasculitis that involves multiple organ systems; the type of support and care depends on the type of organ system involvement. Consultation with a rheumatologist is generally indicated. Specific organ-system involvement may require consultation with one or more of the following specialists:

  • Pulmonologist, for the management of hemoptysis and respiratory failure secondary to pulmonary alveolar capillaritis
  • Cardiologist, for management of myocardial infarction, myocarditis, and heart failure
  • Nephrologist, for the management of renal failure
  • Gastroenterologist
  • Neurologist

Long-Term Monitoring

Eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome) cases must be followed up very closely at a rheumatology clinic or another specialty clinic. Patients with this syndrome usually need long-term immunosuppressive medications.

The patient's clinical status, erythrocyte sedimentation rate, and eosinophil count should be monitored. In EGPA patients with antineutrophil cytoplasmic antibodies (ANCAs), the ANCA level does not correlate well with disease activity. [56]