EGPA and lungs

Q: The involvement of lungs and no other organs is a good prognostic sign in Eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss)?

A) True

B) False

Answer: A

EGPA is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis of the small and medium-sized arteries.

Although lung is the most commonly involved organ in EGPA, also known as Churg-Strauss disease, vasculitis of extrapulmonary organs is mostly responsible for morbidity and mortality. In fact, the most commonly used score, the Five-factor score (FFS), to determine disease activity does not even involve lungs. It counts older age, heart, GI, and kidney involvement but absence of ENT symptoms. The presence of ENT symptoms and involvement of only the lungs are considered good prognostic signs. 

Said that recently (2021) American College of Rheumatology/Vasculitis Foundation (ACR/VF) guidelines recommends approaching the initial treatment of EGPA by differentiating patients into severe and non-severe disease. If pulmonary hemorrhage is present, it predispose patients to severe disease.

The absence of ANCA and minimal symptoms are also good prognostic signs.

#vasculitis

#rheumatology

References:

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 Care Res (Hoboken) 2021; 73:1088.

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.