This 72yo female was on pentosan polysulfate sodium (PPS, Elmiron) for 5-10 years in the 1990s and was previously misdiagnosed with dry age-related macular degeneration (AMD).
While her fundus exam may resemble pigmentary changes associated with dry AMD, there are no drusen.
Fundus autofluorescence (FAF) shows a mixture of hyper- and hypo-autofluorescent spots in the macula extending outwards into the mid-peripheries. Drusen and pigmentary changes in AMD typically exhibit variable hypoautofluorescence.
OCT shows some variable attenuation of the EZ band along with hyperreflective deposits above the RPE.
Learning Points:
PPS, sold under the brand name Elmiron, is used to treat interstitial cystitis. PPS maculopathy seems to develop over many years and can mimic more common disorders, including AMD and macular dystrophies. As with hydroxychloroquine, macular changes can progress despite discontinuation of the drug. In our experience, wide-field FAF is critical in establishing the diagnosis.
Although there is no treatment for the underlying maculopathy, patients need to be followed and treated if macular neovascularization develops.
