PR3ANCA Related Cerebral Vasculitis in Ulcerative Colitis Presenting with Orbital Involvement: A Case Report with Review of Literature.

Athira Unnikrishnan,Nadeem Ansari,Shila Azodi,Robert C Uchiyama,Joshua Kamnetz,Megan Brown

doi:10.1155/2014/582094

Abstract

PR3 ANCA is a classic marker of granulomatosis with polyangiitis (GPA). There have been several recent reports of increased prevalence of PR3ANCA in ulcerative colitis (UC) patients, the clinical implication of which is not well defined. We are reporting a case of 27-year-old Caucasian male with 14-year history of UC presenting with unilateral proptosis, conjunctival congestion, and chemosis who developed acute hemiparesis within three days of hospital admission, followed by rapid neurological deterioration correlating with brain imaging findings. Serologically he had atypical PANCA with high PR3 antibody titer with a negative infectious workup. His cerebral angiogram was normal but the brain biopsy showed necrotizing vasculitis. He was diagnosed with PR3 ANCA mediated cerebral and orbital vasculitis associated with UC. Treatment was initiated with high dose steroids, plasmapheresis, and cyclophosphamide. He improved significantly with residual left hemiparesis.

Highlights

PR3 ANCA is a classic marker of granulomatosis with polyangiitis (GPA)
Our case of cerebral vasculitis associated with ulcerative colitis is the fourth histopathologically proven case in the literature
There are no reports of orbital vasculitis and cerebral vasculitis coexisting in a patient with ulcerative colitis

Summary

Introduction

PR3 ANCA is a classic marker of granulomatosis with polyangiitis (GPA). There have been several recent reports of increased prevalence of PR3ANCA in ulcerative colitis patients, the clinical implication of which is not well defined [1]. In small cohort studies PR3 ANCA has been associated with extensive colitis and shorter disease duration without any gender predilection [2]. The incidence of small vessel vasculitis in PR3 ANCA positive UC patient cohort is not known. We are reporting the first case of PR3ANCA associated fulminant cerebral vasculitis in an ulcerative colitis patient

Case Summary

Discussion

Conclusion