Derrick Wang1, Swetha B Velaga2, Christelle Grondin1, Adrian Au1, Muneeswar Nittala2, Jay Chhablani3, Kiran K Vupparaboina3, Frederic Gunnemann1, JooYeon Jung1, Ja-Hong Kim4, Michael Ip5, SriniVas Sadda5, David Sarraf6. 1. Retinal Disorders and Ophthalmic Genetics, Stein Eye Institute, University of California Los Angeles (D.W., C.G., A.A., F.G., J.Y.J., D.S.), Los Angeles, California, USA. 2. Doheny Image Reading Center, Doheny Eye Institute (S.B.V., M.N., M.I., S.V.S.), Los Angeles, California, USA. 3. Department of Ophthalmology, UPMC Eye Center, University of Pittsburgh (J.C., K.K.V.), Pittsburgh, Pennsylvania, USA. 4. Department of Urology, UCLA Medical Center, David Geffen School of Medicine at UCLA (J.H.K.), Los Angeles, California, USA. 5. Doheny Image Reading Center, Doheny Eye Institute (S.B.V., M.N., M.I., S.V.S.), Los Angeles, California, USA; Department of Ophthalmology, David Geffen School of Medicine at University of California Los Angeles (M.I., S.V.S.), Los Angeles, California, USA. 6. Retinal Disorders and Ophthalmic Genetics, Stein Eye Institute, University of California Los Angeles (D.W., C.G., A.A., F.G., J.Y.J., D.S.), Los Angeles, California, USA; Greater Los Angeles VA Healthcare Center (D.S.), Los Angeles, California, USA. Electronic address: dsarraf@ucla.edu.
Abstract
PURPOSE: To describe the prevalence and spectrum of disease of pentosan polysulfate (PPS) maculopathy in a large multimodal retinal imaging study and to report the results of choroidal vascularity index (CVI) analysis. DESIGN: Prospective cohort study Methods: Of 741 patients prescribed PPS within a large university database, 100 (13.4%) with any consumption agreed to participate in a prospective screening investigation. Multimodal retinal imaging including near-infrared reflectance (NIR), fundus autofluorescence (FAF), and spectral domain optical coherence tomography (SD-OCT) was performed in all patients. Characteristic findings of affected patients were identified, and affected and unaffected cohorts were compared. CVI, defined as stromal choroidal area (SCA) divided by the total choroidal area, was analyzed. RESULTS: The prevalence of PPS maculopathy was 16%. NIR illustrated punctate hyperreflective lesions with early presentation. FAF illustrated a speckled macular network of hypo- and hyperautofluorescence colocalized with multifocal hyperreflective retinal pigment epithelial lesions on SD-OCT. Advanced cases demonstrated varying degrees of atrophy. The affected cohort exhibited significantly greater mean PPS therapy duration, mean daily dosage, and mean cumulative dosage (19.5±5.5 years, 433.9±137.6 mg, 3,103.1±1,402.2 g) compared with the unaffected cohort (7.1±6.6 years, 291.6±177.6 mg, 768.4±754.8 g). SCA was significantly lower and CVI was significantly greater in the affected vs the unaffected group. CONCLUSIONS: This prospective cohort study identified a prevalence of PPS maculopathy of 15%-20% among PPS users who agreed to participate. A spectrum of findings may be observed with multimodal retinal imaging. Significant choroidal abnormalities associated with this characteristic maculopathy may provide surrogate markers of macular toxicity. Published by Elsevier Inc.
PURPOSE: To describe the prevalence and spectrum of disease of pentosan polysulfate (PPS) maculopathy in a large multimodal retinal imaging study and to report the results of choroidal vascularity index (CVI) analysis. DESIGN: Prospective cohort study Methods: Of 741 patients prescribed PPS within a large university database, 100 (13.4%) with any consumption agreed to participate in a prospective screening investigation. Multimodal retinal imaging including near-infrared reflectance (NIR), fundus autofluorescence (FAF), and spectral domain optical coherence tomography (SD-OCT) was performed in all patients. Characteristic findings of affected patients were identified, and affected and unaffected cohorts were compared. CVI, defined as stromal choroidal area (SCA) divided by the total choroidal area, was analyzed. RESULTS: The prevalence of PPS maculopathy was 16%. NIR illustrated punctate hyperreflective lesions with early presentation. FAF illustrated a speckled macular network of hypo- and hyperautofluorescence colocalized with multifocal hyperreflective retinal pigment epithelial lesions on SD-OCT. Advanced cases demonstrated varying degrees of atrophy. The affected cohort exhibited significantly greater mean PPS therapy duration, mean daily dosage, and mean cumulative dosage (19.5±5.5 years, 433.9±137.6 mg, 3,103.1±1,402.2 g) compared with the unaffected cohort (7.1±6.6 years, 291.6±177.6 mg, 768.4±754.8 g). SCA was significantly lower and CVI was significantly greater in the affected vs the unaffected group. CONCLUSIONS: This prospective cohort study identified a prevalence of PPS maculopathy of 15%-20% among PPS users who agreed to participate. A spectrum of findings may be observed with multimodal retinal imaging. Significant choroidal abnormalities associated with this characteristic maculopathy may provide surrogate markers of macular toxicity. Published by Elsevier Inc.