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

| Peer-Reviewed

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

Views: Downloads:

Download PDF

Share This Article

Twitter
Linked In
Facebook

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), 2024. Published by Science Publishing Group

Keywords

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

References

[1]	Kuwana M, Kaburaki J, Okano Y et al. Clinical and prognostic associations based on serum antinuclear antibodies in Japanese patients with systemic sclerosis. Arthritis Rheum. 1994; 37: 75-83.
[2]	Hesselstrand R, Scheja A, Shen GQ et al. The association of antinuclear antibodies with organ involvement and survival in systemic sclerosis. Rheumatology. 2003; 42: 534-40.
[3]	Ashton N, Coomes EN, Garner A et al. Retinopathy due to progressive systemic sclerosis. J Pathol Bacteriol. 1968; 96: 259-68.
[4]	Farkas TG, Sylvester V, Archer D. The choroidopathy of progressive systemic sclerosis (scleroderma). Am J Ophthalmol. 1972; 74: 875-86.
[5]	Littlejohn GO, Urowitz MB, Pavlin CJ. Central retinal vein occlusion and scleroderma: implications for sclerodermatous vascular disease. Ann Rheum Dis. 1981; 40: 96-9.
[6]	Busquets J, Lee Y, Santamarina L et al. Acute retinal artery occlusion in systemic sclerosis: a rare manifestation of systemic sclerosis fibroproliferative vasculopathy. Semin Arthritis Rheum. 2013; 43: 204-8.
[7]	Rotondo C, Lopalco G, Lapadula G et al. Retinal vessels thrombosis as onset manifestation of systemic sclerosis: 3 clinical cases. J Rheumatol. 2014; 41: 2495-6.
[8]	Saari K, Rudenberg H, Laitinen O. Bilateral central retinal vein occlusion in a patient with scleroderma. Ophthalmologica. 1981; 182: 7-12.
[9]	Konuk O, Ozdek S, Onal B et al. Ocular ischemic syndrome presenting as central retinal artery occlusion in scleroderma. Retina. 2006; 26: 102-4.
[10]	Raja MS, Marshall T, Burton BJ. Acute central retinal artery occlusion presenting as CREST syndrome: a case report. Cases J. 2009; 2: 9-12.
[11]	Malik F, Habash AA. Presentation of acute central retinal vein occlusion in scleroderma. Saudi J Ophthalmol. 2015; 29: 156-9.
[12]	Desai SR, Veeraraghavan S, Hansell DM et al. CT features of lung disease in patients with systemic sclerosis: Comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Radiology. 2004; 232: 560-7.
[13]	van den Hoogen F, Khanna D, Fransen J et al. 2013 Classification criteria for systemic sclerosis: An ACR-EULAR collaborative initiative. Arthritis Rheum. 2013; 65: 2737-47.
[14]	Laszik ZG, Silva FG. Systemic sclerosis (systemic scleroderma). In: Jennette JC, Olson JL, Schwartz MM, Silva FG, editors. Heptinstall’s Pathology of the Kidney, Sixth edition. Philadelphia: Lippincott Williams & Wilkins; 2006: P. 730-40.
[15]	Butler EA, Baron M, Fogo AB et al. Generation of a Core Set of Items to Develop Classification Criteria for Scleroderma Renal Crisis Using Consensus Methodology. Arthritis Rheumatol. 2019; 71: 964-71.
[16]	Hayreh SS, Zimmerman MB. Central retinal artery occlusion: visual outcome. Am J Ophthalmol. 2005; 140: 376-91.
[17]	Jorizzo PA, Klein ML, Shults WT et al. Visual recovery in combined central retinal artery and central retinal vein occlusion. Am J Ophthalmol. 1987; 104: 358-63.
[18]	Batal I, Domsic RT, Medsger TA et al. Scleroderma renal crisis: A pathology perspective. Int J Rheumatol. 2010; 2010: 543704.
[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.
[20]	Ubukata M, Mitsuhashi A, Nishizawa Y et al. Anticentromere antibody-positive scleroderma renal crisis requiring dialysis. Intern Med. 2018; 57: 3479-83.
[21]	Jacquier M, Mousson C, Rebibou J et al. Scleroderma renal crisis in a systemic sclerosis with anti-PM/Scl antibodies. Kidney Int Reports. 2019; 4: 1499-502.
[22]	Carrero JL, Novoa Sanjurjo FJ. Bilateral cilioretinal artery occlusion in antiphospholipid syndrome. Retina. 2006; 26: 104-6.
[23]	Chu ER, Chen CS. Concurrent central retinal artery occlusion and branch retinal vein occlusion in giant cell arteritis. Clin Ophthalmol. 2010; 4: 565-7.
[24]	Frech TM, Revelo MP, Drakos SG et al. Vascular leak is a central feature in the pathogenesis of systemic sclerosis. J Rheumatol. 2012; 39: 1385-91.
[25]	Müller-Ladner U, Distler O, Ibba-Manneschi L et al. Mechanisms of vascular damage in systemic sclerosis. Autoimmunity. 2009; 42: 587-95.
[26]	Salojin KV, Le Tonquèze M, Saraux A, et al. Antiendothelial cell antibodies: useful markers of systemic sclerosis. Am J Med. 1997; 102: 178-85.
[27]	Kahaleh MB, Fan PS. Mechanism of serum-mediated endothelial injury in scleroderma: identification of a granular enzyme in scleroderma skin and sera. Clin Immunol Immunopathol. 1997; 83: 32-40.

Cite This Article

Plain Text BibTeX RIS

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

Copy | Download

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

Copy | Download

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  -

Copy | Download

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

Download PDF

Sections

Plain Text BibTeX RIS

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

Copy | Download

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

Copy | Download

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  -

Copy | Download