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Case Reports in Rheumatology
Volume 2014, Article ID 582094, 6 pages
http://dx.doi.org/10.1155/2014/582094
Case Report

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

1Department of Internal Medicine, University of Texas Southwestern Medical School Residency Programs at Austin, University Medical Center Brackenridge, Austin, TX 78701, USA
2Department of Neurology, University of Texas Southwestern Medical School Residency Programs at Austin, University Medical Center Brackenridge, Austin, TX 78701, USA
3Transitional Program, University of Texas Southwestern Medical School Residency Programs at Austin, University Medical Center Brackenridge, Austin, TX 78701, USA
4School of Medicine, University of Texas Medical Branch, Galveston, TX 77555, USA

Received 21 April 2014; Accepted 5 June 2014; Published 2 July 2014

Academic Editor: Tsai-Ching Hsu

Copyright © 2014 Athira Unnikrishnan et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.