Cases of anti–aquaporin4 antibody–positive neuromyelitis optica spectrum disorder treated with eculizumab initiated immediately after acute treatment

Abstract

In November 2019, eculizumab (ECZ) became available in Japan for the prevention of recurrence of anti–aquaporin (AQP)–4 antibody–positive neuromyelitis optica spectrum disorder (NMOSD). However, a complement is involved in its acute pathological condition, and the drug is expected to be effective in the acute phase. Here, we report two cases of anti–AQP4 antibody–positive NMOSD wherein anti–complement therapy was initiated immediately after the end of acute treatment, with the expectation of improvement of acute pathology and prevention of recurrence. The clinical improvement trend continued after the initiation of ECZ, which was a useful treatment strategy. Since the effect of the single agent has not been evaluated, caution is required while interpreting the results. However, to start anti–complement therapy early after relapse may be useful treatment strategy in anti–AQP4 antibody–positive NMOSD. A verification study is expected in the future.