Eosinophilic granulomatosis with polyangiitis (EGPA) or Churg Strauss syndrome

Eosinophilic granulomatosis with polyangiitis (EGPA), or traditionally termed Churg Strauss syndrome was first described in 1951 by Churg and Strauss. It is a rare primary systemic necrotizing vasculitis affecting small and medium sized vessels .Perinuclear antineutrophil cytoplasmic antibody (p-ANCA) is positive in up to 40%–50% of cases [1]. This multisystem disease is typically characterized by 3 phases which include prodromal phase (with atopic disease, allergic rhinitis, and bronchial asthma), eosinophilic phase (peripheral blood eosinophilia or eosinophilic infiltration of multiple organs) and a vasculitic phase of which constitutional symptoms is a presage. Asthma and lung disease are the cardinal clinical features of EGPA and are present in more than 90% of the cases. Neurological involvement in EGPA is rare. Even in the presence of infrequent involvement of CNS in EGPA, it is usually seen in the later phase of the disease [1]
. CNS is affected in 6% to 10% of patients with EGPA and it causes encephalopathy, ischemic infarcts or haemorrhages. (Murthy et al.,
2013)[2].This is a unique case of a patient with EGPA presenting with pan CNS and gut involvement at the diagnosis without lung or upper airway

Author: CeylonMediweb