Bilateral ischaemic retinal vasculopathy in scleroderma

Minasian, Margaret

doi:10.1136/bjo.2004.061499

Cited by 14 publications

(7 citation statements)

References 5 publications

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“…Minasian et al reported CWSs in a normotensive scleroderma patient, though the additional findings included bilateral disc neovascularization and marked vascular tortuosity. 9 Usiyama et al studied retinal findings in normotensive SSc patients and found hard exudates and vascular tortuosities. 10 Our case similarly demonstrates vascular tortuosity.…”

Section: Discussion/conclusionsupporting

confidence: 88%

A case of systemic sclerosis/lupus overlap syndrome presenting with bilateral cotton wool spots

Liles

Warner

Warwar

2020

American Journal of Ophthalmology Case Reports

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Purpose Systemic sclerosis (SSc) is characterized by multi-system inflammation and fibrosis. Ophthalmologists must be aware of the uncommon ocular features of SSc to secure the diagnosis. Observations Here we report the rare occurrence of bilateral cotton wool spots in an 86-year-old woman with SSc/lupus overlap syndrome presenting with a history of chronic obstructive pulmonary disease, gastroesophageal reflux disease, polymyalgia rheumatica, scalp tenderness, and right jaw pain on chewing. Fundoscopy showed diffuse bilateral cotton wool spots that prompted the diagnosis of SSc/lupus overlap syndrome. Conclusions The confluence of patient symptoms was disguised as separate diseases, but the funduscopic finding of cotton wool spots in a patient without known risk factors prompted further investigation and the correct diagnosis. She was started on immunosuppressant therapy but unfortunately died four months later after developing right heart failure. Importance The differential diagnosis for bilateral cotton wool spots should include autoimmune processes such as SSc and systemic lupus erythematosus and may represent an early sign that can help direct treatment.

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Section: Discussion/conclusionsupporting

confidence: 88%

A case of systemic sclerosis/lupus overlap syndrome presenting with bilateral cotton wool spots

Liles

Warner

Warwar

2020

American Journal of Ophthalmology Case Reports

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“…Involvement of the posterior segment of the eye is often subclinical 6,7. Reported ophthalmoscopic findings in patients with SSc include microvascular changes, cotton-wool spots, exudates, cystoid bodies, retinal and optic nerve head edema, hemorrhages, and retinal vein and artery occlusion 8,17,47,48,49. These abnormalities appear to be more typical of advanced SSc with renal involvement and hypertension 8,17.…”

Section: Discussionmentioning

confidence: 99%

Ocular findings in patients with systemic sclerosis

Gomes

Santhiago

Magalhães

et al. 2011

Clinics

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OBJECTIVE: To evaluate the frequency and characteristics of ocular manifestations in outpatients with systemic sclerosis. METHODS: In this cross-sectional study, 45 patients with systemic sclerosis were enrolled. Data regarding demographics, disease duration and subtype, age at diagnosis, nailfold capillaroscopic pattern and autoantibody profile were collected, and a full ophthalmic examination was conducted. Parametric (Student's t-test) and nonparametric (Mann-Whitney U test) tests were used to compare continuous variables. Fisher's exact test was used to compare categorical data. P values < 0.05 were considered significant. RESULTS: Twenty-three subjects (51.1%) had eyelid skin changes; 22 (48.9%) had keratoconjunctivitis sicca, 19 (42.2%) had cataracts, 13 (28.9%) had retinal microvascular abnormalities and 6 (13.3%) had glaucoma. Eyelid skin changes were more frequent in patients with the diffuse subtype of systemic sclerosis and were associated with a younger age and an earlier age at diagnosis. Cataracts were presumed to be age-related and secondary to corticosteroid treatment. There was no association between demographic, clinical or serological data and keratoconjunctivitis sicca. The retinal microvascular abnormalities were indistinguishable from those related to systemic hypertension and were associated with an older age and a severe capillaroscopic pattern. CONCLUSIONS: Eyelid skin abnormalities and keratoconjunctivitis sicca were the most common ocular findings related to systemic sclerosis. Some demographic and clinical data were associated with some ophthalmic features and not with others, showing that the ocular manifestations of systemic sclerosis are characterized by heterogeneity and reflect the differences in the implicated pathophysiological mechanisms.

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“…Although the effects of vascular abnormalities and dysfunction in patients with SSc are most dramatic when they involve the pulmonary and renal arterioles, causing renal crisis [25, 26] and pulmonary artery hypertension [27–29], respectively, there are numerous other important clinical manifestations of the disease that are caused or mediated by the prominent fibroproliferative vasculopathy. These include capillary rarefaction and capillary loop dilation in the nailfold capillaries [30, 31], cutaneous and mucosal telangiectasias [32–34], erectile dysfunction resulting from alterations in penile blood flow [35–37], and cardiac dysfunction including nonartherosclerotic myocardial infarcts [38, 39], gastric antral vascular ectasia [40–42], central retinal artery occlusion [43, 44], and involvement of larger vessels [45, 46]. Histopathologically, the affected vessels display marked narrowing or even complete occlusion of the vessel lumen with remarkable accumulation of mesenchymal cells and fibrous tissue in the subendothelial compartment and associated endothelial cell abnormalities, which include swelling and apoptotic changes, as well as thickening of the basement membrane.…”

Section: Vascular Abnormalities In Sscmentioning

confidence: 99%

Role of Endothelial to Mesenchymal Transition in the Pathogenesis of the Vascular Alterations in Systemic Sclerosis

2013

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The pathogenesis of Systemic Sclerosis (SSc) is extremely complex, and despite extensive studies, the exact mechanisms involved are not well understood. Numerous recent studies of early events in SSc pathogenesis have suggested that unknown etiologic factors in a genetically receptive host trigger structural and functional microvascular endothelial cell abnormalities. These alterations result in the attraction, transmigration, and accumulation of immune and inflammatory cells in the perivascular tissues, which in turn induce the phenotypic conversion of endothelial cells and quiescent fibroblasts into activated myofibroblasts, a process known as endothelial to mesenchymal transition or EndoMT. The activated myofibroblasts are the effector cells responsible for the severe and frequently progressive fibrotic process and the fibroproliferative vasculopathy that are the hallmarks of SSc. Thus, according to this hypothesis the endothelial and vascular alterations, which include the phenotypic conversion of endothelial cells into activated myofibroblasts, play a crucial role in the development of the progressive fibrotic process affecting skin and multiple internal organs. The role of endothelial cell and vascular alterations, the potential contribution of endothelial to mesenchymal cell transition in the pathogenesis of the tissue fibrosis, and fibroproliferative vasculopathy in SSc will be reviewed here.

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Bilateral ischaemic retinal vasculopathy in scleroderma

Cited by 14 publications

References 5 publications

A case of systemic sclerosis/lupus overlap syndrome presenting with bilateral cotton wool spots

A case of systemic sclerosis/lupus overlap syndrome presenting with bilateral cotton wool spots

Ocular findings in patients with systemic sclerosis

Role of Endothelial to Mesenchymal Transition in the Pathogenesis of the Vascular Alterations in Systemic Sclerosis

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