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Purtscher-like Retinopathy in a Patient with Systemic Sclerosis: A Case Report and Narrative Review
Purtscher-like retinopathy (PLR) is an uncommon occlusive microangiopathy associated with various systemic conditions. We report a case of PLR related to severe progressive systemic sclerosis (SSc), an autoimmune disease characterized by widespread angiopathy and fibrosis, in a 44-year-old Caucasian...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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MDPI
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10044861/ https://www.ncbi.nlm.nih.gov/pubmed/36979818 http://dx.doi.org/10.3390/biomedicines11030839 |
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author | Pieklarz, Barbara Gińdzieńska-Sieśkiewicz, Ewa Zawadzka, Izabela Bagrowska, Magdalena Daniluk, Joanna Konopińska, Joanna Kowal-Bielecka, Otylia Dmuchowska, Diana Anna |
author_facet | Pieklarz, Barbara Gińdzieńska-Sieśkiewicz, Ewa Zawadzka, Izabela Bagrowska, Magdalena Daniluk, Joanna Konopińska, Joanna Kowal-Bielecka, Otylia Dmuchowska, Diana Anna |
author_sort | Pieklarz, Barbara |
collection | PubMed |
description | Purtscher-like retinopathy (PLR) is an uncommon occlusive microangiopathy associated with various systemic conditions. We report a case of PLR related to severe progressive systemic sclerosis (SSc), an autoimmune disease characterized by widespread angiopathy and fibrosis, in a 44-year-old Caucasian male diagnosed with early diffuse cutaneous systemic sclerosis (dSSc). Upon ophthalmological examination, pathognomonic fundoscopy abnormalities were found. Spectral domain optical coherence tomography (SD-OCT), angio-OCT, and visual field results are documented at initial diagnosis and follow-up visits. The detailed ophthalmological assessment is juxtaposed with rheumatological evaluation and treatment. Current literature on probable pathophysiological mechanisms is reviewed in accordance with the described case. The PLR seems to be connected to severe SSc-related angiopathy initiated by capillary endothelial damage, with ultimate arteriolar precapillary occlusion in the inner retinal layer. Although this is not routinely recommended, we suggest that ophthalmological examinations may be advantageous in patients with SSc, as serious eye pathology may be present despite the lack of symptoms reported by the patient. Patients with PLR require a differential diagnosis and regular follow-up. Proper treatment of the underlying disease may have beneficial effects on the natural course of PLR. |
format | Online Article Text |
id | pubmed-10044861 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-100448612023-03-29 Purtscher-like Retinopathy in a Patient with Systemic Sclerosis: A Case Report and Narrative Review Pieklarz, Barbara Gińdzieńska-Sieśkiewicz, Ewa Zawadzka, Izabela Bagrowska, Magdalena Daniluk, Joanna Konopińska, Joanna Kowal-Bielecka, Otylia Dmuchowska, Diana Anna Biomedicines Case Report Purtscher-like retinopathy (PLR) is an uncommon occlusive microangiopathy associated with various systemic conditions. We report a case of PLR related to severe progressive systemic sclerosis (SSc), an autoimmune disease characterized by widespread angiopathy and fibrosis, in a 44-year-old Caucasian male diagnosed with early diffuse cutaneous systemic sclerosis (dSSc). Upon ophthalmological examination, pathognomonic fundoscopy abnormalities were found. Spectral domain optical coherence tomography (SD-OCT), angio-OCT, and visual field results are documented at initial diagnosis and follow-up visits. The detailed ophthalmological assessment is juxtaposed with rheumatological evaluation and treatment. Current literature on probable pathophysiological mechanisms is reviewed in accordance with the described case. The PLR seems to be connected to severe SSc-related angiopathy initiated by capillary endothelial damage, with ultimate arteriolar precapillary occlusion in the inner retinal layer. Although this is not routinely recommended, we suggest that ophthalmological examinations may be advantageous in patients with SSc, as serious eye pathology may be present despite the lack of symptoms reported by the patient. Patients with PLR require a differential diagnosis and regular follow-up. Proper treatment of the underlying disease may have beneficial effects on the natural course of PLR. MDPI 2023-03-10 /pmc/articles/PMC10044861/ /pubmed/36979818 http://dx.doi.org/10.3390/biomedicines11030839 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Case Report Pieklarz, Barbara Gińdzieńska-Sieśkiewicz, Ewa Zawadzka, Izabela Bagrowska, Magdalena Daniluk, Joanna Konopińska, Joanna Kowal-Bielecka, Otylia Dmuchowska, Diana Anna Purtscher-like Retinopathy in a Patient with Systemic Sclerosis: A Case Report and Narrative Review |
title | Purtscher-like Retinopathy in a Patient with Systemic Sclerosis: A Case Report and Narrative Review |
title_full | Purtscher-like Retinopathy in a Patient with Systemic Sclerosis: A Case Report and Narrative Review |
title_fullStr | Purtscher-like Retinopathy in a Patient with Systemic Sclerosis: A Case Report and Narrative Review |
title_full_unstemmed | Purtscher-like Retinopathy in a Patient with Systemic Sclerosis: A Case Report and Narrative Review |
title_short | Purtscher-like Retinopathy in a Patient with Systemic Sclerosis: A Case Report and Narrative Review |
title_sort | purtscher-like retinopathy in a patient with systemic sclerosis: a case report and narrative review |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10044861/ https://www.ncbi.nlm.nih.gov/pubmed/36979818 http://dx.doi.org/10.3390/biomedicines11030839 |
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