Prognosis

The outcome in EGPA is dependent on the extent and severity of organ involvement. In most cases, with appropriate immunosuppressive therapy, survival seems comparable to age-matched controls. The survival rates are better than those associated with the other antineutrophil cytoplasmic antibody vasculitides, with published 5-year survival rates of 68% to 100%.[14][15] Myocardial and central nervous system (CNS) involvement may confer greatest risk.

Five-factor score (FFS) of poor prognostic indicators in polyarteritis nodosa and EGPA

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

  • Proteinuria >1 g/24 hours

  • Serum creatinine >1.58 mg/dL

  • Gastrointestinal tract involvement

  • Cardiomyopathy

  • CNS involvement

When none of the five factors are present (FFS = 0), 5-year mortality has been reported at 11.9%. Mortality increases as the number of these five factors increases, with mortality reported at 49.95% when at least two factors are present (FFS ≥2).[37] This scoring system has use in determining prognosis in polyarteritis nodosa as well.

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 EGPA (n=230), granulomatosis with polyangiitis (formerly known as Wegener 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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