Abstract General Information

Title

Therapeutic plasma exchange for neuromyelitis optica attacks: evidence and challenges from a real-world cohort from Brazil

Abstract

Background

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease that presents with demyelinating attacks, which can lead to blindness or paraplegia. Therapeutic plasma exchange (TPE) is associated with better neurological improvement after NMOSD attacks. However, real-world data on TPE in Latin America is lacking. Our goal was to evaluate response and safety of TPE for NMOSD attacks in a cohort from Brazil.

Method

We conducted a retrospective cohort study of all consecutive patients admitted between January 2010 and December 2018 in Neurology clinic - Hospital das Clínicas São Paulo, Brazil. Inclusion criteria were acute demyelinating attacks treated with TPE in patients fullfilling IPND 2015 diagnosis criteria of NMOSD. Aquaporin-4 (AQP4-IgG) in the serum by cell-based assay (CBA) was done in all patients. Clinical response was classified as absent, mild, moderate, or marked improvement upon discharge, and after six to twelve months. Patients with moderate and marked improvement were considered TPE responders. We evaluated adverse events, the characteristics of the NMOSD attacks as prognostic factors for response.

Results

We evaluated 84 admissions for NMOSD attacks in 68 patients. 61 out of 68 (89.7%) of our sample were women and the median age was 40 (29, 48). AQP4-IgG was positive in 53/68 (77.9%) of patients. Median interval between symptoms onset and TPE was 25 days (13, 49). In 55 out of 84 (65.5%) attacks, patients were considered TPE responders on discharge. Adverse events occurred in 39% of treatments, particularly arterial hypotension (15%) and central line-associated bloodstream infection (CLABSI) (7 per 1000 catheter days). Delayed TPE did not impact significantly in responders.

Conclusion

In this retrospective study, TPE was effective to treat NMOSD attacks in most patients. Delayed TPE was not associated with clinical response. Bloodstream infection remains a challenge and CLABSI may be improved with better techniques and reinforce nurse care.

Area

NMO, ADEM and CIS