Because NMOSD is an inflammatory autoimmune syndrome of the CNS with devastating clinical outcomes, adequate treatment of early attacks is decisive to prevent severe disability. The first-line treatment for acute attacks is high-dose intravenous steroid therapy. Therapeutic plasma exchange and immunoadsorption serve to remove plasma components involved in the inflammation. IVIG has been used for the treatment of a number of autoimmune disorders with various efficacy, but has not produced consistent results for acute attacks in immune-mediated demyelinating diseases. Currently, IVIG is considered empirically as an alternative and/or additional treatment for acute attacks of NMOSD after steroids and plasma exchange fail to offset symptoms or are contraindicated.
This retrospective study by Dr De-Cai Tian (Capital Medical University, China) investigated the efficacy of IVIG for patients with NMOSD during acute attacks [1]. A total of 198 patients were included; 85% was female, mean age at onset was 38 years, median EDSS at onset was 5.0. In these patients, 243 attacks occurred, classified as isolated acute myelitis (50.6%), isolated optic neuritis (9.9%), simultaneous acute myelitis and optic neuritis (14.0%), or other (25.5%). The following treatments were given:
- high-dose intravenous steroids in 153 attacks;
- IVIG in 14 attacks;
- episodes of IVIG plus high-dose intravenous steroids in 69 attacks; and
- plasma exchange in 7 attacks.
Of those attacks treated with IVIG alone, 35.7% showed a moderate decrease of EDSS score 33%-65%, while the other attacks alleviated poorly (<33% decrease). Significantly fewer patients treated with IVIG alone had better outcomes compared with patients who received high-dose intravenous steroids alone (P=0.004). No significant difference was found between IVIG plus high-dose steroids compared with steroids alone (P=0.073). When stratified by EDSS score at onset (≤6.0 versus ≥6.5), sequential treatments of IVIG in addition to high-dose steroids increased the likelihood of clinical improvement in severe attacks for patients with higher EDSS score at onset, compared with steroids alone (P=0.040).
- Li X, et al. Intravenous immunoglobulin for acute attacks in neuromyelitis optica spectrum disorders. ECF 28th Annual Meeting. Abstract 79.
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