Eosinophilic granulomatosis with polyangiitis with an unusual presentation

• Autor(a) principal: Nogueira,Rui Filipe
• Data de Publicação: 2021
• Outros Autores: Belmira,Ana, Sousa,Vitor, Alves,Rui
• Tipo de documento: Relatório
• Idioma: eng
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000200118
• topic: Acute Kidney Failure; Eosinophilic Granulomatosis with Polyangiitis; Interstitial Nephritis; Kidney Biopsy.

ABSTRACT Eosinophilic granulomatosis with polyangiitis is an ANCA vasculitis characterized by asthma, rhinosinusitis and peripheral eosinophilia. The kidney is infrequently involved, usually in the form of necrotizing crescentic glomerulonephritis. We present the case of a 60-year-old man who presented with painless sudden loss of visual acuity, purpuric exanthem in his legs, asthenia and myalgia. CT-scan ruled out acute vascular and intracranial space occupant lesions. Optical coherence tomography showed signs of left central retinal artery occlusion and perfusion deficits in the right arterial retinal blood supply. Complementary study showed prominent peripheral eosinophilia (24.500 cel/uL), increased serum IgE (1260U/L) and increased C-reactive protein (10.6mg/dl). During admission, the patient presented with acute kidney failure (serum creatinine of 4.7mg/dl) and an exceptionally high p-ANCA MPO titer (>600U/L). Eosinophilic granulomatosis with polyangiitis was diagnosed and plasmapheresis, pulse steroid therapy and intravenous cyclophosphamide were provided. Kidney biopsy showed interstitial nephritis with high eosinophil content while the glomerulus was relatively spared, with only mild endocapillary proliferation. The patient didn’t require dialysis. Kidney function was normal at discharge, although the visual deficit did not improve.