Abstract

We report a 62-year-old Caucasian lady who presented with a progressive history of unsteadiness, vertigo, falls and cognitive decline. MRI brain and spinal cord showed multifocal white matter lesions and diffuse meningeal enhancement. A diagnosis of sarcoidosis was made based on brain biopsy. Over the next 2 years, she received prednisolone with methotrexate followed by cyclophosphamide. She continued to deteriorate. Infliximab was started but her condition worsened and she eventually died. Post mortem pathology was compatible with necrotising sarcoid granulomatosis (NSG). NSG is a rare diagnosis. We discuss the clinical and pathological similarities between sarcoidosis and NSG. The refractoriness to infliximab is of interest in this case as it is usually effective in sarcoidosis. We discuss the possible explanations for this such as the development of antibodies against infliximab and the possibility that NSG is a separate entity. An increased awareness of this specific histological diagnosis is likely to lead to a better understanding of its prognosis and treatment options.