Central Retinal Arterial and Venous Occlusion in a Patient with Systemic Sclerosis and a Review of the Related Literature

Aya Mori; Kimimasa Nakabayashi; Hiroko Konishi; Ippei Doi; Rie Tagaya; Aya Nakase; Masayoshi Iwakami

doi:doi:10.11648/j.ajim.20210904.19

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Central Retinal Arterial and Venous Occlusion in a Patient with Systemic Sclerosis and a Review of the Related Literature

Aya Mori, Kimimasa Nakabayashi, Hiroko Konishi, Ippei Doi, Rie Tagaya, Aya Nakase, Masayoshi Iwakami

Published in American Journal of Internal Medicine (Volume 9, Issue 4)

Received: 14 July 2021 Accepted: 5 August 2021 Published: 27 August 2021

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Abstract

Systemic sclerosis (SSc) is known to cause serious complications at an advanced stage. However, serious ocular complications such as central retinal artery occlusion (CRAO) and/or central retinal vein occlusion (CRVO) have only been reported in a few cases to date. A 51-year-old man with severe Raynaud’s phenomenon, scleroderma up to the forearm and a negative anti-nuclear antibody (ANA) finding developed pulmonary fibrosis (PF) and was admitted to the hospital for the treatment of dyspnea under the diagnosis of SSc. A transbronchial lung biopsy performed 10 days after admission demonstrated mucoid intimal thickening (MIT) of the small arteries in the peripheral lung tissue and the bronchial wall. Prednisolone (PSL), ciclosporin and nintedanib were prescribed for PF treatment but failed to induce any apparent improvement in dyspnea. Finally, methylprednisolone pulse therapy (1.0 g, 3 times) and intravenous cyclophosphamide (IVCY, 500 mg, 6 times) therapy were instituted with a subsequent decrease in the patient’s Krebs von den Lungen 6 (KL-6) and surfactant protein D (SP-D) levels. During IVCY therapy, the patient experienced blurred vision in the right eye, and this was diagnosed as being due to CRAO and CRVO. However, the patient did not manifest any embolic, coagulopathic, or vasculitic symptoms, and there were no abnormal laboratory data suggesting these diseases in relation to this symptom. Therefore, the ocular occlusion was presumed to be due to MIT of small arteries, in addition to severe Raynaud’s phenomenon. Thus, we report the findings of this important case, despite the fact that it lacks a retinal pathology.

Published in	American Journal of Internal Medicine (Volume 9, Issue 4)
DOI	10.11648/j.ajim.20210904.19
Page(s)	214-218
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2021. Published by Science Publishing Group

Keywords

Systemic Sclerosis, Central Retinal Artery Occlusion, Raynaud’s Phenomenon, Pulmonary Fibrosis, Mucoid Intimal Thickening

References

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[19]	Trostle DC, Bedetti CD, Steen VD et al. Renal vascular histology and morphometry in systemic sclerosis. A case-control autopsy study. Arthritis Rheum. 1988; 31: 393-400.
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APA Style

Aya Mori, Kimimasa Nakabayashi, Hiroko Konishi, Ippei Doi, Rie Tagaya, et al. (2021). Central Retinal Arterial and Venous Occlusion in a Patient with Systemic Sclerosis and a Review of the Related Literature. American Journal of Internal Medicine, 9(4), 214-218. https://doi.org/10.11648/j.ajim.20210904.19

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ACS Style

Aya Mori; Kimimasa Nakabayashi; Hiroko Konishi; Ippei Doi; Rie Tagaya, et al. Central Retinal Arterial and Venous Occlusion in a Patient with Systemic Sclerosis and a Review of the Related Literature. Am. J. Intern. Med. 2021, 9(4), 214-218. doi: 10.11648/j.ajim.20210904.19

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AMA Style

Aya Mori, Kimimasa Nakabayashi, Hiroko Konishi, Ippei Doi, Rie Tagaya, et al. Central Retinal Arterial and Venous Occlusion in a Patient with Systemic Sclerosis and a Review of the Related Literature. Am J Intern Med. 2021;9(4):214-218. doi: 10.11648/j.ajim.20210904.19

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@article{10.11648/j.ajim.20210904.19,
  author = {Aya Mori and Kimimasa Nakabayashi and Hiroko Konishi and Ippei Doi and Rie Tagaya and Aya Nakase and Masayoshi Iwakami},
  title = {Central Retinal Arterial and Venous Occlusion in a Patient with Systemic Sclerosis and a Review of the Related Literature},
  journal = {American Journal of Internal Medicine},
  volume = {9},
  number = {4},
  pages = {214-218},
  doi = {10.11648/j.ajim.20210904.19},
  url = {https://doi.org/10.11648/j.ajim.20210904.19},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajim.20210904.19},
  abstract = {Systemic sclerosis (SSc) is known to cause serious complications at an advanced stage. However, serious ocular complications such as central retinal artery occlusion (CRAO) and/or central retinal vein occlusion (CRVO) have only been reported in a few cases to date. A 51-year-old man with severe Raynaud’s phenomenon, scleroderma up to the forearm and a negative anti-nuclear antibody (ANA) finding developed pulmonary fibrosis (PF) and was admitted to the hospital for the treatment of dyspnea under the diagnosis of SSc. A transbronchial lung biopsy performed 10 days after admission demonstrated mucoid intimal thickening (MIT) of the small arteries in the peripheral lung tissue and the bronchial wall. Prednisolone (PSL), ciclosporin and nintedanib were prescribed for PF treatment but failed to induce any apparent improvement in dyspnea. Finally, methylprednisolone pulse therapy (1.0 g, 3 times) and intravenous cyclophosphamide (IVCY, 500 mg, 6 times) therapy were instituted with a subsequent decrease in the patient’s Krebs von den Lungen 6 (KL-6) and surfactant protein D (SP-D) levels. During IVCY therapy, the patient experienced blurred vision in the right eye, and this was diagnosed as being due to CRAO and CRVO. However, the patient did not manifest any embolic, coagulopathic, or vasculitic symptoms, and there were no abnormal laboratory data suggesting these diseases in relation to this symptom. Therefore, the ocular occlusion was presumed to be due to MIT of small arteries, in addition to severe Raynaud’s phenomenon. Thus, we report the findings of this important case, despite the fact that it lacks a retinal pathology.},
 year = {2021}
}

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TY  - JOUR
T1  - Central Retinal Arterial and Venous Occlusion in a Patient with Systemic Sclerosis and a Review of the Related Literature
AU  - Aya Mori
AU  - Kimimasa Nakabayashi
AU  - Hiroko Konishi
AU  - Ippei Doi
AU  - Rie Tagaya
AU  - Aya Nakase
AU  - Masayoshi Iwakami
Y1  - 2021/08/27
PY  - 2021
N1  - https://doi.org/10.11648/j.ajim.20210904.19
DO  - 10.11648/j.ajim.20210904.19
T2  - American Journal of Internal Medicine
JF  - American Journal of Internal Medicine
JO  - American Journal of Internal Medicine
SP  - 214
EP  - 218
PB  - Science Publishing Group
SN  - 2330-4324
UR  - https://doi.org/10.11648/j.ajim.20210904.19
AB  - Systemic sclerosis (SSc) is known to cause serious complications at an advanced stage. However, serious ocular complications such as central retinal artery occlusion (CRAO) and/or central retinal vein occlusion (CRVO) have only been reported in a few cases to date. A 51-year-old man with severe Raynaud’s phenomenon, scleroderma up to the forearm and a negative anti-nuclear antibody (ANA) finding developed pulmonary fibrosis (PF) and was admitted to the hospital for the treatment of dyspnea under the diagnosis of SSc. A transbronchial lung biopsy performed 10 days after admission demonstrated mucoid intimal thickening (MIT) of the small arteries in the peripheral lung tissue and the bronchial wall. Prednisolone (PSL), ciclosporin and nintedanib were prescribed for PF treatment but failed to induce any apparent improvement in dyspnea. Finally, methylprednisolone pulse therapy (1.0 g, 3 times) and intravenous cyclophosphamide (IVCY, 500 mg, 6 times) therapy were instituted with a subsequent decrease in the patient’s Krebs von den Lungen 6 (KL-6) and surfactant protein D (SP-D) levels. During IVCY therapy, the patient experienced blurred vision in the right eye, and this was diagnosed as being due to CRAO and CRVO. However, the patient did not manifest any embolic, coagulopathic, or vasculitic symptoms, and there were no abnormal laboratory data suggesting these diseases in relation to this symptom. Therefore, the ocular occlusion was presumed to be due to MIT of small arteries, in addition to severe Raynaud’s phenomenon. Thus, we report the findings of this important case, despite the fact that it lacks a retinal pathology.
VL  - 9
IS  - 4
ER  -

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Author Information

Aya Mori

Department of Internal Medicine, Kugayama Hospital, Tokyo, Japan
Kimimasa Nakabayashi

Department of Internal Medicine, Kugayama Hospital, Tokyo, Japan
Hiroko Konishi

Department of Ophthalmology, Kugayama Hospital, Tokyo, Japan
Ippei Doi

Department of Radiology, Kugayama Hospital, Tokyo, Japan
Rie Tagaya

Department of Internal Medicine, Kugayama Hospital, Tokyo, Japan
Aya Nakase

Department of Internal Medicine, Kugayama Hospital, Tokyo, Japan
Masayoshi Iwakami

Department of Internal Medicine, Kugayama Hospital, Tokyo, Japan

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APA Style

Aya Mori, Kimimasa Nakabayashi, Hiroko Konishi, Ippei Doi, Rie Tagaya, et al. (2021). Central Retinal Arterial and Venous Occlusion in a Patient with Systemic Sclerosis and a Review of the Related Literature. American Journal of Internal Medicine, 9(4), 214-218. https://doi.org/10.11648/j.ajim.20210904.19

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ACS Style

Aya Mori; Kimimasa Nakabayashi; Hiroko Konishi; Ippei Doi; Rie Tagaya, et al. Central Retinal Arterial and Venous Occlusion in a Patient with Systemic Sclerosis and a Review of the Related Literature. Am. J. Intern. Med. 2021, 9(4), 214-218. doi: 10.11648/j.ajim.20210904.19

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AMA Style

Aya Mori, Kimimasa Nakabayashi, Hiroko Konishi, Ippei Doi, Rie Tagaya, et al. Central Retinal Arterial and Venous Occlusion in a Patient with Systemic Sclerosis and a Review of the Related Literature. Am J Intern Med. 2021;9(4):214-218. doi: 10.11648/j.ajim.20210904.19

Copy | Download

@article{10.11648/j.ajim.20210904.19,
  author = {Aya Mori and Kimimasa Nakabayashi and Hiroko Konishi and Ippei Doi and Rie Tagaya and Aya Nakase and Masayoshi Iwakami},
  title = {Central Retinal Arterial and Venous Occlusion in a Patient with Systemic Sclerosis and a Review of the Related Literature},
  journal = {American Journal of Internal Medicine},
  volume = {9},
  number = {4},
  pages = {214-218},
  doi = {10.11648/j.ajim.20210904.19},
  url = {https://doi.org/10.11648/j.ajim.20210904.19},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajim.20210904.19},
  abstract = {Systemic sclerosis (SSc) is known to cause serious complications at an advanced stage. However, serious ocular complications such as central retinal artery occlusion (CRAO) and/or central retinal vein occlusion (CRVO) have only been reported in a few cases to date. A 51-year-old man with severe Raynaud’s phenomenon, scleroderma up to the forearm and a negative anti-nuclear antibody (ANA) finding developed pulmonary fibrosis (PF) and was admitted to the hospital for the treatment of dyspnea under the diagnosis of SSc. A transbronchial lung biopsy performed 10 days after admission demonstrated mucoid intimal thickening (MIT) of the small arteries in the peripheral lung tissue and the bronchial wall. Prednisolone (PSL), ciclosporin and nintedanib were prescribed for PF treatment but failed to induce any apparent improvement in dyspnea. Finally, methylprednisolone pulse therapy (1.0 g, 3 times) and intravenous cyclophosphamide (IVCY, 500 mg, 6 times) therapy were instituted with a subsequent decrease in the patient’s Krebs von den Lungen 6 (KL-6) and surfactant protein D (SP-D) levels. During IVCY therapy, the patient experienced blurred vision in the right eye, and this was diagnosed as being due to CRAO and CRVO. However, the patient did not manifest any embolic, coagulopathic, or vasculitic symptoms, and there were no abnormal laboratory data suggesting these diseases in relation to this symptom. Therefore, the ocular occlusion was presumed to be due to MIT of small arteries, in addition to severe Raynaud’s phenomenon. Thus, we report the findings of this important case, despite the fact that it lacks a retinal pathology.},
 year = {2021}
}

Copy | Download

TY  - JOUR
T1  - Central Retinal Arterial and Venous Occlusion in a Patient with Systemic Sclerosis and a Review of the Related Literature
AU  - Aya Mori
AU  - Kimimasa Nakabayashi
AU  - Hiroko Konishi
AU  - Ippei Doi
AU  - Rie Tagaya
AU  - Aya Nakase
AU  - Masayoshi Iwakami
Y1  - 2021/08/27
PY  - 2021
N1  - https://doi.org/10.11648/j.ajim.20210904.19
DO  - 10.11648/j.ajim.20210904.19
T2  - American Journal of Internal Medicine
JF  - American Journal of Internal Medicine
JO  - American Journal of Internal Medicine
SP  - 214
EP  - 218
PB  - Science Publishing Group
SN  - 2330-4324
UR  - https://doi.org/10.11648/j.ajim.20210904.19
AB  - Systemic sclerosis (SSc) is known to cause serious complications at an advanced stage. However, serious ocular complications such as central retinal artery occlusion (CRAO) and/or central retinal vein occlusion (CRVO) have only been reported in a few cases to date. A 51-year-old man with severe Raynaud’s phenomenon, scleroderma up to the forearm and a negative anti-nuclear antibody (ANA) finding developed pulmonary fibrosis (PF) and was admitted to the hospital for the treatment of dyspnea under the diagnosis of SSc. A transbronchial lung biopsy performed 10 days after admission demonstrated mucoid intimal thickening (MIT) of the small arteries in the peripheral lung tissue and the bronchial wall. Prednisolone (PSL), ciclosporin and nintedanib were prescribed for PF treatment but failed to induce any apparent improvement in dyspnea. Finally, methylprednisolone pulse therapy (1.0 g, 3 times) and intravenous cyclophosphamide (IVCY, 500 mg, 6 times) therapy were instituted with a subsequent decrease in the patient’s Krebs von den Lungen 6 (KL-6) and surfactant protein D (SP-D) levels. During IVCY therapy, the patient experienced blurred vision in the right eye, and this was diagnosed as being due to CRAO and CRVO. However, the patient did not manifest any embolic, coagulopathic, or vasculitic symptoms, and there were no abnormal laboratory data suggesting these diseases in relation to this symptom. Therefore, the ocular occlusion was presumed to be due to MIT of small arteries, in addition to severe Raynaud’s phenomenon. Thus, we report the findings of this important case, despite the fact that it lacks a retinal pathology.
VL  - 9
IS  - 4
ER  -

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