Posterior reversible encephalopathy syndrome and digital gangrene in a patient with granulomatosis with polyangiitis : a rare case report

Abstract

Granulomatosis with Polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis(AAV), classically associated with the presence of proteinase 3 (PR3)-ANCA. GPA has a wide range of clinical manifestations, with multiorgan involvement, particularly upper and lower respiratory tract, and kidneys. One of itsrarest manifestationsis digital ischemia or gangrene that accounts for <1% of cases. Posterior reversible posterior leukoencephalopathy syndrome (PRES) is a syndrome characterized by headache, visual changes, confusion, and seizures, accompanied by distinctive neuroimaging features, which are usually reversible. Rare cases of its association with vasculitis, including GPA, have been described. Others conditions such as end-stage renal disease, immunosuppressive drug use and eclampsia are additional risk factors for the development of PRES.