36.: A rare case of central nervous system vasculitis requiring infliximab to achieve remission

Granulomatous vasculitis of the central nervous system (CNS) is an uncommon illness characterised by sporadic small to medium sized vessel involvement of the meninges, brain and spinal cord. It presents with CNS dysfunction and is often difficult to diagnose due to a wide range of differential diagnoses and occasionally non-definitive investigations. The prognosis is poor unless long term remission is achieved via immunosuppression. We illustrate a rare case of granulomatous vasculitis of the CNS where the patient failed conventional first and second line immunosuppressive therapies, eventually achieving remission using the tumour necrosis factor (TNF)-α blocker, infliximab. A previously well 34-year-old man first presented in 2009 with ataxia and nausea, accompanied by lethargy/malaise. Initial MRI of the brain revealed diffuse signal abnormality within the white matter of the cerebellum as well as a patchy, diffuse nodular leptomeningeal enhancement in the posterior fossa. Cerebrospinal fluid examination was abnormal with elevated protein of 1.7 g/L, approximately 100 mononuclear cells, polymerase chain reaction was negative for tuberculosis and herpes simplex virus, and glucose/cytology was unremarkable. Screening tests for systemic vasculitis were unremarkable including an angiotensin-converting enzyme level of 16. Formal angiogram showed no evidence of vasculitis, however a leptomeningeal biopsy revealed a granulomatous vasculitis with no specific diagnostic features. Typical therapies such as cyclophosphamide, pulse dose methylprednisolone, methotrexate, azathioprine, mycophenolate and rituximab all failed. Remission was eventually achieved with a combination of infliximab and oral prednisolone. This case adds further to a small number of case reports where TNF-α blockade has been used effectively to treat multi drug resistant vasculitis of the CNS. Despite the fact that randomised control trial data is lacking in this subject due to the low incidence of the disease and difficulties in diagnosis, TNF blockade appears a viable option in treatment resistant cases.