Evaluation of prognostic factors for patients with eosinophilic granulomatosis with polyangiitis recruited at the pneumonological centre and mainly ANCA negativity: A retrospective analysis of a single cohort in Poland

Abstract

ABSTRACT Objectives The aim was to investigate the risk factors for relapse and death in patients with eosinophilic granulomatosis with polyangiitis (EGPA) recruited at the pneumonological centre and mainly antineutrophil cytoplasmic antibody negativity. Methods We retrospectively recruited 86 patients. Relapse was defined as the recurrence or appearance of new organ symptoms. The study end-point included the final examination. Results Relapses occurred in 34.9% of the patients, while 9.3% died. Immunosuppressive therapy (P = 0.042), prolonged low-dose corticosteroid treatments (mainly for asthma) (P = 0.006), and longer follow-up duration (P = 0.004) were associated with a higher relapse risk, while advanced EGPA severity (P = 0.0015) and activity (P = 0.044), older age of onset (P = 0.030), symptomatic cardiac involvement (P = 0.007), and postinflammatory cardiac fibrosis (P = 0.038) were associated with a higher risk of death. Sinusitis (P = 0.028) and prolonged low-dose corticosteroid treatments (P = 0.025) correlated with a better prognosis. Relapses did not have an impact on the mortality (P = 0.693). Conclusions Relapses in EGPA remain frequent, although they do not impact mortality. Cardiac involvement is common, but clinically symptomatic cardiomyopathy is associated with a higher risk of death. Asthma requiring chronic corticosteroid treatments is associated with a lower risk of death, although the risk of EGPA recurrence is significantly higher.