Interleukin-6 in neuromyelitis optica spectrum disorder pathophysiology

Kazuo Fujihara, Jeffrey L. Bennett, Jerome de Seze, Masayuki Haramura, Ingo Kleiter, Brian G. Weinshenker, Delene Kang, Tabasum Mughal, Takashi Yamamura

Research output: Contribution to journalReview articlepeer-review

9 Scopus citations


Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder that preferentially affects the spinal cord and optic nerve. Most patients with NMOSD experience severe relapses that lead to permanent neurologic disability; therefore, limiting frequency and severity of these attacks is the primary goal of disease management. Currently, patients are treated with immunosuppressants. Interleukin-6 (IL-6) is a pleiotropic cytokine that is significantly elevated in the serum and the CSF of patients with NMOSD. IL-6 may have multiple roles in NMOSD pathophysiology by promoting plasmablast survival, stimulating the production of antibodies against aquaporin-4, disrupting blood-brain barrier integrity and functionality, and enhancing proinflammatory T-lymphocyte differentiation and activation. Case series have shown decreased relapse rates following IL-6 receptor (IL-6R) blockade in patients with NMOSD, and 2 recent phase 3 randomized controlled trials confirmed that IL-6R inhibition reduces the risk of relapses in NMOSD. As such, inhibition of IL-6 activity represents a promising emerging therapy for the management of NMOSD manifestations. In this review, we summarize the role of IL-6 in the context of NMOSD.

Original languageEnglish (US)
JournalNeurology(R) neuroimmunology & neuroinflammation
Issue number5
StatePublished - Sep 3 2020

ASJC Scopus subject areas

  • Medicine(all)


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