Criteria

There are no clinical diagnostic criteria. The criteria and definition below were established for research purposes.

American College of Rheumatology/European Alliance of Associations for Rheumatology 2022 classification criteria for EGPA[2]

These criteria should be applied to classify a patient as having EGPA when a diagnosis of small- or medium-vessel vasculitis has been made.

Clinical criteria

  • Obstructive airway disease (+3)

  • Nasal polyps (+3)

  • Mononeuritis multiplex (+1)

Laboratory and biopsy criteria

  • Blood eosinophil count ≥1 x 10⁹/L (+5)

  • Extravascular eosinophilic-predominant inflammation on biopsy (+2)

  • Positive test for cytoplasmic anti-neutrophil cytoplasmic antibodies (cANCA) or anti-proteinase 3 (anti-PR3) antibodies (-3)

  • Haematuria (-1)

A score of ≥6 is needed for the classification of vasculitis as EGPA.

Chapel Hill consensus conference definition of eosinophilic granulomatosis with polyangiitis[1]

An eosinophil-rich and granulomatous inflammation involving the respiratory tract, and necrotising vasculitis affecting small- to medium-sized vessels, and associated with asthma and eosinophilia.

Five-factor score (FFS) of poor prognostic indicators in polyarteritis nodosa and eosinophilic granulomatosis with polyangiitis[37]

This scoring system allows for the prediction of prognosis of patients with EGPA vasculitis. Presence of any of the five factors below contributes to mortality and outcome.

  • Proteinuria >1 g/24 hours

  • Serum creatinine >1.58 mg/dL (>140 micromol/L)

  • Gastrointestinal tract involvement

  • Cardiomyopathy

  • Central nervous system involvement

When none of the five factors are present (FFS = 0), 5-year mortality is 11.9%. Mortality increases as the number of these five factors increases, with mortality increasing to 49.95% when at least two factors are present (FFS ≥2). This scoring system has use in determining prognosis in polyarteritis nodosa as well.

Five-factor score (FFS) revisited: assessment of prognoses of systemic necrotising vasculitides based on the French Vasculitis Study Group cohort[38]

A revised 2009 FFS has been published.[38] The original cohort of 342 patients in whom the initial FFS was developed was extended to include 1108 patients with either eosinophilic granulomatosis with polyangiitis (n=230), granulomatous polyangiitis (formerly known as Wegener's granulomatosis) (n=311), microscopic polyangiitis (n=218), or polyarteritis nodosa (n=349). This updated FFS has four negative prognostic factors (-1):

  • Age >65 years

  • Cardiac insufficiency

  • Renal insufficiency

  • Gastrointestinal tract involvement

and one positive prognostic factor (+1):

  • Ear, nose, and throat involvement.

This new scoring system has not yet been evaluated in clinical treatment trials.

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