Abstract

Overview, aims and methods: EGPA is a rare disease associated with eosinophilic vasculitis and severe asthma. The advances in treatment with biologicals, Mepolizumab (Wechsler et al. 2017) have rekindled the interest. We reviewed our patients' case records.

Results: 6 patients, 3 F, 3 M, age range between 17 to 64. 5/6 non-smokers had EGPA. All had asthma, eosinophilia >10% and absolute eosinophil count >1, paranasal sinusitis from CT and or nasal endoscopy. 5 had pulmonary infiltrates, 3 had nasal polyps, 4 had mono-neuritis. Pathological proof of vasculitis was obtained in two by nasal mucosal biopsies,  2 had biopsy proved extravascular eosinophilic infiltration, (lung, bone marrow). p-ANCA (MPO)was positive in 3(50%). All 6 met ACR criteria for EGPA.(Jennette J C et al. 2013)  Latency from asthma to EGPA ranged between 5 to 15 years.  All had at least one admission for asthma and 5/6 either experienced 3 or more exacerbations in previous 12 months or were on long term steroids prior to diagnosis. 4/6 were commenced on Mepolizumab 300 mg once every 4 weeks. Other 2 were managed on pulse steroids and immunosuppression. One year or more of post Mepolizumab, all have lower ACQ scores, no exacerbations and achieved a significant reduction in maintenance steroid dose.

Conclusion: EGPA is difficult to diagnose.The current point/period prevalence in Jersey islands stands at 60 per million person- years, more than many European countries. Once commenced on biological, there is a reduction in exacerbations, maintenance steroid dose and improved asthma control. Awareness of EGPA needs to be increased.