FFS-EGPA

Q: Presence of ear, nose, and throat (ENT) symptoms predict poor outcome in Eosinophilic Granulomatosis with PolyAngiitis (EGPA) (Churg-Strauss)?

A) True

B) False

Answer: B

Sounds strange but the presence of ENT symptoms predicts a better prognosis in EGPA, also known as Churg-Strauss syndrome. 

EGPA is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis of the small and medium-sized arteries. The severity of the disease can be calculated by various scores but one of the commonly used score in clinical setting is "five-factor score" (FFS). The score was initially proposed in 1996 and was revised in 2011. It includes,

• Age >65
• Cardiac insufficiency
• Gastrointestinal (GI) involvement
• Renal insufficiency (stabilized peak plasma creatinine concentration >1.7 mg/dL [150 micromol/L])
• Absence of ENT manifestations 

#rheumatology

References:

Guillevin L, Pagnoux C, Seror R, et al. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore) 2011; 90:19.

Gayraud M, Guillevin L, le Toumelin P, et al. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum 2001; 44:666.

Moosig F, Bremer JP, Hellmich B, et al. A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients. Ann Rheum Dis 2013; 72:1011.