Aseptic Meningitis With Multifocal White Matter T2 Hyperintensity in a Patient with Ulcerative Colitis on Vedolizumab: A Case Report
INTRODUCTION: Progressive multifocal leukoencephalopathy (PML) is a rare but serious complication of receiving natalizumab, an ?4 integrin inhibitor. No case report of PML has been reported with vedolizumab, a gut-specific anti-?4?7-integrin monoclonal antibody. We describe a case of aseptic meningitis in a patient with ulcerative colitis on vedolizumab who was found to have multifocal white matter T2 hyperintensity on brain MRI.
CASE DESCRIPTION: A 25-year-old man presented as an outside hospital transfer for further workup of recurrent fever of unknown origin. His past medical history is notable for well controlled ulcerative pan-colitis on vedolizumab. He has been on vedolizumab every 8 weeks for the past 2 years and he received the most recent dose 2 months prior to admission. In the past 6 months, he had symptoms of cyclical fevers in addition to occasional headaches and shoulder pain. On presentation, he was febrile and tachycardic with physical exam notable for an erythematous, blanching macular rash on his chest. Laboratory studies demonstrated an elevated erythrocyte sedimentation rate of 79 mm/Hr, C-reactive protein of 50 mg/L, fecal calprotectin of 502 mcg/g, and white blood cell count (WBC) of 18.6*103/uL. Computed tomography of the abdomen demonstrated mild thickening of the proximal colon, diffuse lymphadenopathy and hepatosplenomegaly. On the sixth day of admission, the patient developed severe headache and vasodilatory shock requiring norepinephrine and vasopressin in the intensive care unit. Lumbar puncture was performed, which demonstrated a WBC of 3305 (84% neutrophils), protein of 323 and glucose <20. He underwent a brain magnetic resonance imaging that demonstrated multiple foci of T2 hyperintensity involving subcortical white matter concerning for PML or other acute demyelinating processes. Despite a positive JC virus IgG antibody, the cerebrospinal fluid (CSF) was negative for JC virus based on polymerase chain reaction (PCR) as well as other bacterial, fungal or viral infections. After extensive negative infectious workup, patient was started on 1mg/kg intravenous methylprednisolone for treatment of Adult-onset Still’s disease (AOSD), since he met both Yamaguchi and Fautrel’s criteria. The diagnosis of AOSD was also supported by his abnormally elevated levels of IL-6 and IL-18. He was discharged with oral steroids and started on Anakinra (IL-1 receptor antagonist) during outpatient follow-up. His recurrent fevers and headaches have resolved and his CRP has downtrended to 10 at the most recent clinic visit.
DISCUSSION: This is the first reported case of aseptic meningitis complicated by vasodilatory shock secondary to AOSD in the IBD population. Despite patient’s MRI findings was concerning for PML in the setting of a positive serum JC virus antibody, the negative CSF PCR further ensures the safety profile of vedolizumab. AOSD is a diagnosis of exclusion and is characterized by classic triad of daily recurrent fever, salmon colored rash and transient arthritis. CNS involvement in AOSD is rare, but research has suggested 8% of AOSD patients may have CNS involvement, characterized by neutrophilic pleocytosis. We suspect his shock and brain MRI findings were likely due to systemic cytokine release from AOSD.
