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2017 ; 12
(1
): 129
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Efficacy of rituximab in non-paraneoplastic autoimmune retinopathy
#MMPMID28709429
Boudreault K
; Justus S
; Sengillo JD
; Schuerch K
; Lee W
; Cabral T
; Tsang SH
Orphanet J Rare Dis
2017[Jul]; 12
(1
): 129
PMID28709429
show ga
BACKGROUND: Autoimmune retinopathy (AIR) is a rare but potentially blinding
condition that is often underdiagnosed. Common features in AIR presentation
include rapidly progressive vision loss with abnormal electrophysiological
responses of the retina associated with positive anti-retinal antibodies. AIR is
also challenging to treat, and thus, the introduction of new potential
therapeutic agents is welcomed. The goal of this communication is to assess the
effects of rituximab infusions on electroretinogram (ERG) responses and visual
function outcomes in patients with non-paraneoplastic autoimmune retinopathy
(npAIR). RESULTS: Following infusion(s), three out of five patients showed no
evidence of disease progression or improved, while two patients continued to
progress on ERG. One patient demonstrated improvement in visual acuity (2 lines)
in both eyes. ERG responses provided objective monitoring of patients' visual
function and response to immunosuppression over time. CONCLUSIONS: These findings
suggest that patients with npAIR unresponsive to other immunosuppression
therapies may benefit from rituximab infusion, although stabilization rather than
improvement was more frequently the outcome in our case series. Furthermore,
regularly scheduled ERG follow-up examinations are recommended for monitoring
patients' progression during treatment.
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