PMID- 29494697 OWN - NLM STAT- MEDLINE DCOM- 20180622 LR - 20181114 IS - 1932-6203 (Electronic) IS - 1932-6203 (Linking) VI - 13 IP - 3 DP - 2018 TI - Sickle cell maculopathy: Identification of systemic risk factors, and microstructural analysis of individual retinal layers of the macula. PG - e0193582 LID - 10.1371/journal.pone.0193582 [doi] LID - e0193582 AB - PURPOSE: To identify systemic risk factors for sickle cell maculopathy, and to analyze the microstructure of the macula of Sickle Cell Disease (SCD) patients by using automated segmentation of individual retinal layers. METHODS: Thirty consecutive patients with SCD and 30 matched controls underwent spectral-domain optical coherence tomography (SD-OCT) and automated thickness measurement for each retinal layer; thicknesses for SCD patients were then compared to normal controls. Demographic data, systemic data, and lab results were collected for each SCD patient; multivariate logistic regression analysis was used to identify potential risk factors for sickle cell maculopathy. RESULTS: Ongoing chelation treatment (p = 0.0187) was the most predictive factor for the presence of sickle cell maculopathy; the odds were 94.2% lower when chelation was present. HbF level tended to influence sickle cell maculopathy (p = 0.0775); the odds decreased by 12.9% when HbF increased by 1%. Sickle cell maculopathy was detected in 43% of SCD patients as patchy areas of retinal thinning on SD-OCT thickness map, mostly located temporally to the macula, especially in eyes with more advanced forms of sickle cell retinopathy (p = 0.003). In comparison to controls, SCD patients had a subtle thinning of the overall macula and temporal retina compared to controls (most p<0.0001), involving inner and outer retinal layers. Thickening of the retinal pigment epithelium was also detected in SCD eyes (p<0.0001). CONCLUSIONS: Chronic chelation therapy and, potentially, high levels of HbF are possible protective factors for the presence of sickle cell maculopathy, especially for patients with more advanced forms of sickle cell retinopathy. A subtle thinning of the overall macula occurs in SCD patients and involves multiple retinal layers, suggesting that ischemic vasculopathy may happen in both superficial and deep capillary plexi. Thinning of the outer retinal layers suggests that an ischemic insult of the choriocapillaris may also occur in SCD patients. FAU - Dell'Arti, Laura AU - Dell'Arti L AD - Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy. AD - Ophthalmological Unit, Ca' Granda Foundation, IRCCS Ospedale Maggiore Policlinico, Milan, Italy. FAU - Barteselli, Giulio AU - Barteselli G AD - Genentech Inc, South San Francisco, California, United States of America. FAU - Riva, Lorenzo AU - Riva L AD - Ophthalmological Unit, Ca' Granda Foundation, IRCCS Ospedale Maggiore Policlinico, Milan, Italy. FAU - Carini, Elisa AU - Carini E AD - Ophthalmological Unit, Ca' Granda Foundation, IRCCS Ospedale Maggiore Policlinico, Milan, Italy. FAU - Graziadei, Giovanna AU - Graziadei G AD - Rare Diseases Center, Department of Medicine and Medical Specialties, Ca' Granda Foundation, IRCCS Ospedale Maggiore Policlinico, Milan, Italy. FAU - Benatti, Eleonora AU - Benatti E AD - Ophthalmological Unit, Ca' Granda Foundation, IRCCS Ospedale Maggiore Policlinico, Milan, Italy. FAU - Invernizzi, Alessandro AU - Invernizzi A AD - Eye Clinic, Department of Biomedical and Clinical Science "L. Sacco", Luigi Sacco Hospital, University of Milan, Milan, Italy. FAU - Cappellini, Maria D AU - Cappellini MD AD - Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy. AD - Rare Diseases Center, Department of Medicine and Medical Specialties, Ca' Granda Foundation, IRCCS Ospedale Maggiore Policlinico, Milan, Italy. FAU - Viola, Francesco AU - Viola F AUID- ORCID: 0000-0003-0533-1135 AD - Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy. AD - Ophthalmological Unit, Ca' Granda Foundation, IRCCS Ospedale Maggiore Policlinico, Milan, Italy. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20180301 PL - United States TA - PLoS One JT - PloS one JID - 101285081 RN - 9034-63-3 (Fetal Hemoglobin) SB - IM MH - Adult MH - Aged MH - Anemia, Sickle Cell/*complications/*drug therapy/metabolism MH - Chelation Therapy/*methods MH - Female MH - Fetal Hemoglobin/metabolism MH - Fluorescein Angiography/methods MH - Humans MH - Macula Lutea/*diagnostic imaging/metabolism MH - Male MH - Middle Aged MH - Odds Ratio MH - Retinal Diseases/*diagnostic imaging/metabolism MH - Risk Factors MH - Tomography, Optical Coherence/methods MH - Young Adult PMC - PMC5832302 COIS- Competing Interests: The authors have read the journal's policy and have the following conflicts: GB is an employee at Genentech Inc. This does not alter our adherence to all the PLOS ONE policies on sharing data and materials. There are no patents, products in developments or marketed products to declare. EDAT- 2018/03/02 06:00 MHDA- 2018/06/23 06:00 PMCR- 2018/03/01 CRDT- 2018/03/02 06:00 PHST- 2017/09/29 00:00 [received] PHST- 2018/02/14 00:00 [accepted] PHST- 2018/03/02 06:00 [entrez] PHST- 2018/03/02 06:00 [pubmed] PHST- 2018/06/23 06:00 [medline] PHST- 2018/03/01 00:00 [pmc-release] AID - PONE-D-17-35209 [pii] AID - 10.1371/journal.pone.0193582 [doi] PST - epublish SO - PLoS One. 2018 Mar 1;13(3):e0193582. doi: 10.1371/journal.pone.0193582. eCollection 2018.