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Show Poster 112 Blurred Margins: Cocaine Use Clouding the Diagnosis of Orbital Granulomatosis With Polyangitis Meari Taguchi1, Poonam Bansal1 1 Henry Ford Neurology, Detroit, Michigan, USA Introduction: Cocaine induced vasculitis and Idiopathic ANCA Associated vasculitis (AAV) share similar presentations which can be difficult to differentiate. We report the case of young woman presenting with right eye vision loss and severe pansinusitis in the setting of cocaine use, who was diagnosed with orbital granulomatosis with polyangiitis. Description of Case(s): A 32 year old woman presented with painless, progressive vision loss in the right eye over a course of 2 weeks. Ophthalmologic examination demonstrated no light perception and abduction deficit in the right eye. Fundoscopy demonstrated 270 degrees of blurred disc margins with frisen grade 2-disc edema. In the left eye visual acuity was 20/20 with normal fundus. Urine drug screen was positive for cocaine. CT sinuses and MRI orbit demonstrated destruction of the nasal septum, middle turbinates, lateral wall of right maxillary and sphenoid sinus. An enhancing soft tissue mass was seen extending through the right inferior orbital fissure into the orbital apex with significant compression/infiltration of right optic nerve. The lateral and medial rectus muscles also appeared enlarged. CT chest demonstrated tiny, non significant opacities. Renal function was normal without proteinuria. Labs included positive C-ANCA 1:20 titer (EIA positive for anti-PR3), negative Levamisole Ab, and negative Human Neutrophil Elastase Ab. Tissue biopsy and pathology demonstrated necrotizing granulomatous inflammation and vasculitis. As the biopsy was most consistent with GPA, patient was started on a lower dose of prednisone at 0.5 mg/kg daily due to concurrent MSSA infection. Conclusions, including unique features of the case(s): A comprehensive review of the clinical presentation, labs, and tissue biopsy is needed to reach diagnosis. Positive UDS for cocaine should not deter the clinician from considering ANCA Associated Vasculitis syndromes. In our case, evidence supporting GPA include minimally elevated Anti-PR3 antibodies, negative levamisole Ab, negative Human Neutrophil Elastase antibody and necrotizing granuloma on biopsy. Orbital involvement can occur in 60% of GPA cases. References: Berman M, Paran D, Elkayam O. Cocaine-Induced Vasculitis. Rambam Maimonides Med J. 2016;7(4):e0036. Published 2016 Oct 31. doi:10.5041/RMMJ.10263 McGrath MM, Isakova T, Rennke HG, Mottola AM, Laliberte KA, Niles JL. Contaminated cocaine and antineutrophil cytoplasmic antibody-associated disease. Clin J Am Soc Nephrol. 2011;6(12):2799-2805. doi:10.2215/CJN.03440411 Muller K, Lin JH. Orbital granulomatosis with polyangiitis (Wegener granulomatosis): clinical and pathologic findings. Arch Pathol Lab Med. 2014;138(8):1110-1114. doi:10.5858/arpa.2013-0006-RS Subesinghe S, van Leuven S, Yalakki L, Sangle S, D'Cruz D. Cocaine and ANCA associated vasculitis-like syndromes - A case series. Autoimmunity Reviews.2018; 17(1):73-77. doi: 10.1016/j.autrev.2017.11.011 Wiesner O, Russell KA, Lee AS, Jenne DE, Trimarchi M, Gregorini G, et al. Antineutrophil cytoplasmic antibodies reacting with human neutrophil elastase as a diagnostic marker for cocaine-induced midline destructive lesions but not autoimmune vasculitis. Arthritis Rheum. 2004;50(9):2954-65. doi:10.1002/art.20479 Keywords: Neuro-ophth & systemic disease (eg. MS, MG, thyroid), Orbit/ocular pathology, Optic neuropathy, Ocular motility, Miscellaneous Financial Disclosures: The authors had no disclosures. Grant Support: None. Contact Information: None provided. 166 | North American Neuro-Ophthalmology Society |
