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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...

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Autores principales: Pieklarz, Barbara, Gińdzieńska-Sieśkiewicz, Ewa, Zawadzka, Izabela, Bagrowska, Magdalena, Daniluk, Joanna, Konopińska, Joanna, Kowal-Bielecka, Otylia, Dmuchowska, Diana Anna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
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.
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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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