Prognosis

EGPA has a favourable prognosis following timely detection and treatment, with a 5-year survival rate of 90%. The relapse rate is estimated at approximately 20% to 30%, often presenting with fever, joint pain, and constitutional symptoms. (1)

Cardiac involvement is the most frequent cause of mortality in patients with poor response to therapy, primarily through myocarditis and coronary arteritis (2)

Factors associated with a poor prognosis include: (3)

• Severe gastrointestinal tract involvement
• Cardiomyopathy
• CNS vasculitis
• Renal failure

The Vasculitis Damage Index (VDI) is a valuable tool for assessing accumulated organ damage resulting from both the disease and its treatment. This tool correlates strongly with mortality and morbidity (4)

References:

1. Mukhtyar C et al. European Vasculitis Study Group (EUVAS). Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League Against Rheumatism systemic vasculitis task force. Ann Rheum Dis. 2008 Jul;67(7):1004-10.
2. Sinico RA et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum. 2005 Sep;52(9):2926-35
3. Nguyen Y, Guillevin L. Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss). Semin Respir Crit Care Med. 2018 Aug;39(4):471-481.
4. Exley AR et al. Examination of disease severity in systemic vasculitis from the novel perspective of damage using the vasculitis damage index (VDI). Br J Rheumatol. 1998 Jan;37(1):57-63