Tocilizumab in Refractory Adult-Onset Still's Disease with Aseptic Meningitis—Efficacy of Interleukin-6 Blockade and Review of the Literature

Adult-onset Still's disease is a multisystem inflammatory disorder of unknown etiology characterized by typical spiking fever, evanescent rash, arthralgia, and leucocytosis. Neurologic manifestations are infrequent, seen in 7 to 12% of cases. We present the case of a young male admitted with aseptic meningitis that satisfied the diagnostic criteria of Adult-onset Still's disease. Refractoriness to therapy with corticosteroids and cyclosporine A led to the use of humanized monoclonal anti-interleukin-6 receptor antibody “tocilizumab” with dramatic response. The case is reported for the rarity of presentation and the need to consider the diagnosis in related clinical scenarios. Also, current literature on the use of tocilizumab in intractable disease is reviewed.

Keywords: adult-onset Still's disease, refractory AOSD, aseptic meningitis, neurologic manifestations, serum ferritin, interleukin-6, tocilizumab