Hodecker SC, Stellmann JP, Rosenkranz SC, Young K, Holst B, Friese MA, Heesen C

Neuromyelitis optica spectrum disorder (NMOSD) is a rare relapsing autoimmune demyelinating disease of the CNS that predominantly affects the optic nerves and the spinal cord.1 Because of the severity and poor recovery of attacks, aggressive immunosuppressive agents are used early in the clinical course to reduce relapse frequency. Apart from classical immunosuppressant agents, biologicals such as rituximab, eculizumab, or tocilizumab have been used for relapse prevention.2 Based on retrospective data, 17–53% of patients have break-through relapses under the most commonly used treatments azathioprine, mycophenolate mofetil, or rituximab.3 While established in myeloproliferative diseases, the JAK/STAT (Janus kinase/signal transducer and activator of transcription) signaling cascade has emerged as a new target for the treatment of autoimmune diseases. JAK inhibitors have proven to be effective in rheumatic diseases.4 Ruxolitinib is an orally available and potent selective inhibitor of the Janus kinases JAK1 and JAK2. Here, we report the case of a patient with highly active neuromyelitis optica who continued to accumulate neurologic disability while receiving numerous immunosuppressive treatments and was ultimately treated with ruxolitinib.

Neurol Neuroimmunol Neuroinflamm. 2017 Feb 3;4(2);e328

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