Eosinophilic granulomatous disease with polyangiitis: clinical case presentation and literature review.

Abstract

Eosinophilic granulomatosis with polyangitis is a systemic necrotizing vasculitis, which involves small and medium sized vessels. It is a rare disease with and incidence and prevalence of 0.5-6 and 7-14 cases per million of habitants per year, respectively. Until now, the etiology remains unknown; genetic and environmental factors have been involved, along with the ANCA and Anti MPO antibodies. The disease evolves in three successive phases: the first stage is characterized by prodromes, with allergic manifestations such as rhinitis, asthma, nasal polyps, among others; a second stage where blood hypereosinophilia and eosinophil tissue infiltrates appear; and the third stage where systemic vasculitis occurs mainly affecting the skin, peripheral nervous system and kidneys. The diagnosis is made on the clinical basis, laboratory findings (eosinophilia and others), antibodies and histopathology. The prognosis is good, but there is high morbidity, the risk of recurrence is 35%. The treatment must be individualized and performed with corticosteroids and cyclophosphamide. We present the case of a 56 years old male patient with history of allergic rhinitis and allergy to betalactamics who develops prolonged fever. Routine lab tests report hyper eosinophilia, later on he develops peripheric neuropathy in upper and lower limbs. Further studies result positive for ANCA and a bone marrow biopsy shows predominance of eosinophilic granules. This patient was treated with oral corticosteroids and pregabalin with evident improvement, nine months after treatment he is in remission and being followed every month.

Keywords: Eosinophilic granulomatosis with polyangitis, antineutrophil cytoplasmic antibody, Hypereosinophilia