Stroke and Vasculitis, a rare but serious complication of Relapsing Polychondritis: A Case Report and Literature Review

Objective: To report a rare case of ischemic stroke associated with vasculitis secondary to relapsing polychondritis (RPC) and review the current literature to report frequency, characteristics, and current management. Background: RPC is an inflammatory disorder of the cartilaginous structures. Ischemic stroke and cerebral vasculitis are extremely rare but have been previously reported. Design/Methods: Case report and literature review via search of PUBMED from 1966 to 2019. English language article titles and abstract were screened, and appropriate articles reviewed. Results: We report a 69-year-old male with a recent diagnosis of RPC not yet on immunosuppressants, who presented with sudden onset of confusion and right side weakness. MRI with contrast showed diffuse leptomeningeal enhancement along the entire neuro-axis, and multi-focal acute and subacute ischemic strokes. Digital Subtraction angiography showed severe intracranial large vessel stenosis and occlusion involving both the anterior and posterior circulation, as well as multi-focal vessel irregularities. The involved vessels had mural enhancement of the vessel wall on 3T MRI. CSF studies showed pleocytosis without any infectious etiology. He was diagnosed with cerebral vasculitis secondary to RPC and treated with high dose steroids with resolution of leptomeningeal enhancement on subsequent MRI. We found 12 cases of ischemic stroke associated with RPC (age range 36–73 years, 60% male) during literature review, of which 7 had cerebral vasculitis diagnosed via cerebral vessel imaging or brain biopsy. The presenting symptoms varied between cases, but all patients presented with confusion, headache and sudden onset of focal neurologic deficits. All patients were treated with corticosteroids or immunosuppressants with improvement or complete resolution of symptoms. Conclusions: Cerebral vasculitis secondary to RPC is a rare but serious condition and has variable clinical manifestations. It can be treated with high-dose steroids or other immunosuppressants. Disclosure: Dr. Chima has nothing to disclose. Dr. Chima has nothing to disclose. Dr. George has nothing to disclose. Dr. Newey has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Bard.