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This is a 42 year old female with a past medical history of GPA resulting in renal failure, post renal transplant in 1999 with recent nephrectomy after rejection, hypertension, and hypothyroidism. She was diagnosed with GPA 15 years prior by renal biopsy and was ANCA +. She was treated with immunosuppression after the renal transplant, but had discontinued medications after having a nephrectomy 3 weeks prior to presentation. She presented to an outside hospital with severe headache and confusion in the setting of systolic blood pressures >200. CT scan showed a subarachnoid hemorrhage involving the basal cisterns and filling the fourth ventricle. Diagnostic angiogram on presentation to our facility was normal with no aneurysms, AVMs or other vascular abnormalities. She was admitted to the NSICU and was treated for hypertensive urgency and had frequent neuro checks. She had severe headache, nausea and vomiting, but no progression of SAH on CT imaging and no need for surgical intervention. On hospital day 5, she developed swelling and new skin lesions on her face. These lesions progressed to involve the extremities, face, and trunk;becoming ulcerated and painful. A rheumatologic work-up was completed and showed: Labs: ANCA screen: negative, ANA: negative. C3: 73 (normal 90-180), C4: 19(normal 10-40). PR3: negative, MPO: positive. Skin biopsy was performed and showed leukocytoclasticvasculitis. In patients with a history of GPA a subarachnoid hemorrhage can be the initial presentation of a vasculitis flare. In GPA 22-54% of patients can present with neurological involvement, most commonly mononeuritismultiplex and polyneuritis while CNS manifestation occurs in only 7-11% of patients. Of those, SAH or ICH are very uncommon. There have only been 8 reported cases of SAH associated with GPA in the literature and only 2 of those cases reported full recovery. SAH in patients with GPA is clinically different than in the general population. All but 1 of the cases of SAH in patients with GPA were non-aneurysmal, while 85% of spontaneous SAH in the general population is caused by ruptured cerebral aneurysms. Another interesting difference is that SAH in the general population has a female preponderance, while only 1 of the 8 previously reported cases in GPA was female


Presented at American College of Physicians New Mexico Chapter Scientific Meeting, 11/07/2014, Albuquerque, NM.