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The patient was successfully treated with rituximab despite being refractory to tocilizumab and corticosteroids. During our patient's clinical course, monitoring cytokine profiles, especially vascular endothelial growth factor, was useful in tracking the disease activity of TAFRO syndrome.Granulomatosis with polyangiitis (GPA) is a rare multisystem autoimmune disease of unknown aetiology with only a few reported cases about spinal cord involvement in the literature. We report on the case of an established GPA presented with acute onset