Abstract
Objective
To describe the prevalence and spectrum of multimodal imaging findings of pentosan
polysulfate sodium (PPS)–associated maculopathy and to recommend dosage-related screening
guidelines.
Design
Cross-sectional study.
Methods
Patients previously or currently treated with PPS at University of California, Los
Angeles, were randomly ascertained and prospectively screened for PPS-associated maculopathy
with multimodal retinal imaging. Daily and cumulative dosages of PPS exposure were
calculated for each patient. Images were studied to identify the characteristic findings
of toxicity. The prevalence of PPS-associated maculopathy and screening guidelines
were determined.
Results
The prevalence of PPS-associated maculopathy in this cohort was 20% (10/50 patients).
Both average duration of PPS therapy and average cumulative dosage were significantly
lower in the unaffected (6.3 ± 6.6 years, 691.7 ± 706.6 g) versus the affected groups
(20.3 ± 6.6 years, 3375.4 ± 1650.0 g, p < 0.001). Near-infrared reflectance (NIR) illustrated characteristic punctate retinal
pigment epithelium (RPE) macular lesions early. Fundus autofluorescence (FAF) showed
speckled autofluorescence in the posterior pole with peripapillary extension. Co-localization
with optical coherence tomography (OCT) displayed focal RPE thickening and, in more
severe cases, RPE atrophy in the macula and even the periphery.
Conclusions
A prevalence of 20% in this study cohort suggests a significant risk of macular toxicity
for PPS-treated patients. Characteristic alterations are best detected with FAF and
NIR. More significant PPS exposure was associated with more severe atrophy. We recommend
an initial baseline eye examination to include OCT and, most importantly, NIR and
FAF with annual retinal imaging thereafter especially with cumulative dosages approaching
500 g. Patients exposed to greater than 1500 g of PPS are at significant risk of retinal
toxicity.
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Article info
Publication history
Published online: January 20, 2020
Accepted:
December 11,
2019
Received in revised form:
December 10,
2019
Received:
November 25,
2019
Identification
Copyright
Published by Elsevier Inc. on behalf of Canadian Ophthalmological Society.
