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

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

Approach Considerations

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 was approved for use in adult patients with EGPA by the US Food and Drug Administration in 2017. 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 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 (OCS) 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. [44]

Next:

Consultations

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

Previous