Pentosan Polysulfate Maculopathy (PPM)
Pathophysiology – toxicity of RPE, choroid and the retina’s extracellular matrix
It is thought that PPS can displace the glycosylated proteins in the extracellular matrix or the interphotoreceptor matrix of the retina similar to how it affects the urothelium.
Additionally, PPS similarity to heparin allows it to also bind to fibroblast growth factors and inhibit signaling pathways needed for retinal growth and regeneration.
OCTA studies showed increased defects on choroidal vascular flow and decreased choroidal stroma in the deeper layers.
Overall, outer retina is affected first in PPM.
Studies show a correlation in increased duration, lower BMI, and increased cumulated dose and development of PPM. Not enough studies have been done to accurately determine the percent of people taking PPS develop PPM and what dose and duration is likely to cause retinal toxicity. However, studies show that PPM can continue to progress even after discontinuation of the drug3,4,5.
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The post Case #74: Pentosan Polysulfate Maculopathy: Another Differential Diagnosis for Night Blindness – Page 14 of 23 appeared first on Retina Revealed.
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