EGPA: diagnosis & treatment stratification

Diagnoses eosinophilic granulomatosis with polyangiitis (Churg-Strauss) using 2022 ACR/EULAR criteria and stratifies treatment intensity with the revised Five-Factor Score. Links the eosinophil count, EGPA classification, and FFS calculators.

Evidence tier: Guideline-derived.

Decision points

  • FFS score, treatment intensity?

Do-not-miss pitfalls

  • Do not diagnose EGPA on eosinophilia + asthma alone, other causes of HES (neoplastic HES, lymphocytic HES) must be excluded.
  • EGPA is ANCA-negative in ~60% of cases, a negative MPO/PR3-ANCA does not exclude EGPA.
  • Cardiac EGPA is underdiagnosed and the leading cause of death, obtain an echocardiogram and cardiac MRI in all newly diagnosed EGPA.
  • Do not stop steroids abruptly after remission, steroid dependence is common and relapse rate is high on taper.
  • Avoid abrupt withdrawal of inhaled corticosteroids (ICS) or leukotriene modifiers before diagnosis, ICS can mask peripheral eosinophilia and delay EGPA recognition.
  • Mepolizumab does not replace corticosteroids for induction in FFS ≥1 disease, it is a maintenance/relapse-prevention agent.
  • Neuropathy in EGPA (mononeuritis multiplex) may not recover even after immunosuppression, set expectations and involve neurology early.

Evidence & citations

  1. Grayson PC, Ponte C, Suppiah R, et al. 2022 American College of Rheumatology / European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis. Ann Rheum Dis. 2022;81(3):309-314. PMID 35110334
  2. Guillevin L, Pagnoux C, Seror R, et al. (French Vasculitis Study Group). The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine. 2011;90(1):19-27. PMID 21200183
  3. Wechsler ME, Akuthota P, Jayne D, et al. (MIRRA study). Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis. N Engl J Med. 2017;376(20):1921-1932. PMID 28514601
  4. Jayne DRW, Merkel PA, Schall TJ, et al. Avacopan for the treatment of ANCA-associated vasculitis (ADVOCATE trial; enrolled GPA/MPA, not EGPA). N Engl J Med. 2021;384(7):599-609. PMID 33596356
  5. Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis Rheumatol. 2021;73(8):1366-1383. PMID 34235894
  6. Benralizumab (Fasenra) FDA approval for eosinophilic granulomatosis with polyangiitis in adults, 18 September 2024, 30 mg SC every 4 weeks; based on the phase 3 MANDARA trial. FDA approval

Clinician decision support. Verify against the cited source. Not a substitute for clinical judgment. 100% on-device; no patient data is stored or transmitted.