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Prevalence of Maculopathy Associated with Long-Term Pentosan Polysulfate Therapy

Published:January 17, 2020DOI:https://doi.org/10.1016/j.ophtha.2020.01.017
      Pentosan polysulfate sodium (PPS) (Elmiron; Janssen Pharmaceuticals, Titusville, NJ) is the only Food and Drug Administration–approved oral medicine for the treatment of interstitial cystitis (IC).
      • Hanno A.M.
      Analysis of long-term Elmiron therapy for interstitial cystitis.
      A recent original report studying 6 patients from a single academic practice linked chronic exposure to PPS with the development of a unique pigmentary maculopathy.
      • Pearce W.A.
      • Chen R.
      • Jain N.
      Pigmentary maculopathy associated with chronic exposure to pentosan polysulfate sodium.
      Further phenotypic details were defined in a larger follow-up retrospective multicenter study involving 35 patients.
      • Hanif A.M.
      • Armenti S.T.
      • Taylor S.
      • et al.
      Phenotypic spectrum of pentosan polysulfate sodium-associated maculopathy.
      Since those reports, presumed PPS-related maculopathy has been noted to progress despite drug cessation
      • Huckfeldt R.M.
      • Vavvas D.
      Progressive maculopathy after discontinuation of pentosan polysulfate sodium.
      and is associated with the development of choroidal neovascularization.
      • Mishra
      • Patel T.P.
      • Singh M.S.
      Choroidal neovascularization associated with pentosan polysulfate toxicity.
      Retrospective studies that have looked at the relationship between PPS intake and maculopathy by reviewing claims databases have provided equivocal results.
      • Jain N.
      • Li A.L.L.
      • Yu Y.
      • VanderBeek B.L.
      Association of macular disease with long-term use of pentosan polysulfate sodium: findings from a US cohort.
      ,
      • Ludwig C.A.
      • Vail D.
      • Callaway N.F.
      • et al.
      Pentosan polysulfate sodium exposure and drug-induced maculopathy in commercially insured patients in the United States.
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