Capillary abnormalities, Raynaud's phenomenon, and systemic sclerosis in patients with localized scleroderma

Maricq, H. R.

doi:10.1001/archderm.128.5.630

Cited by 9 publications

(5 citation statements)

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“…35 A study with 27 adults with localized scleroderma has reported that the only two patients with SD-pattern changes on NFC progressed to SSc. 36 In our study no patient progressed to the systemic form of the disease.…”

Section: Discussionmentioning

confidence: 39%

Capilaroscopia periungueal em crianças e adolescentes com doenças reumáticas

Piotto¹,

Len²,

Hilário³

et al. 2012

Rev. Bras. Reumatol.

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Objective: To assess nailfold capillaroscopy in children and adolescents with autoimmune rheumatic diseases (juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis, scleroderma and mixed connective tissue disease) and relate it to clinical and laboratory fi ndings and disease activity. Methods: Cross-sectional study assessing 147 patients by use of nailfold capillaroscopy as follows: 60 with juvenile idiopathic arthritis; 30 with systemic lupus erythematosus; 30 with juvenile dermatomyositis; 20 with localized scleroderma; four with systemic sclerosis; and three with mixed connective tissue disease. Clinical and laboratory tests and nailfold capillaroscopy were performed in all patients. The nailfold capillaroscopy was performed with an optical microscope (at 10-and 16-time magnifi cations) by the same observer. Results: Most patients (76.2%) had normal nailfold capillaroscopy. The major changes in nailfold capillaroscopy, characterizing the scleroderma pattern, were observed in patients with juvenile dermatomyositis, systemic scleroderma and mixed connective tissue disease. There was no association between nailfold capillaroscopy and disease activity in patients with juvenile idiopathic arthritis, systemic lupus erythematosus and localized scleroderma. Disease activity and capillaroscopy were associated in patients with juvenile dermatomyositis. Conclusion: Nailfold capillaroscopy is a useful method to diagnose autoimmune rheumatic diseases and monitor disease activity.

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Section: Discussionmentioning

confidence: 39%

Capilaroscopia periungueal em crianças e adolescentes com doenças reumáticas

Piotto¹,

Len²,

Hilário³

et al. 2012

Rev. Bras. Reumatol.

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“…Because Raynaud’s phenomenon is a near universal prodrome, there are long-standing suggestions that the initial event in limited scleroderma may affect the vasculature [1]. Even in localized scleroderma, which has fewer systemic features, Raynaud’s progresses to peripheral ischemia with digital ulceration and loss of fingers [2]. Although the vasospasm associated with Raynaud’s phenomenon may be prodromal, it is not itself a sufficient cause of the disease because symptoms of vasospasm can precede diagnosis of limited scleroderma by many years, and the incidence of scleroderma in the population of people with Raynaud’s phenomenon is very low [3•].…”

Section: Introductionmentioning

confidence: 99%

“…These patients develop Raynaud’s phenomenon within 1 year of onset of symptoms but they tend to not have it as a prodrome. Both limited and diffuse scleroderma patients have ischemic changes in their digits (80%–95% have Raynaud’s phenomenon), some with ulcers, gangrene, and loss of fingers [2]. The ischemia is not limited to digits, although this may be the most clinically obvious site.…”

Section: Introductionmentioning

confidence: 99%

Is scleroderma a vasculopathy?

Fleming

Nash²,

Mahoney³

et al. 2009

Curr Rheumatol Rep

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Described as an autoimmune collagen vascular disease, the most striking feature of scleroderma may be a systemic vasculopathy. This vasculopathy includes characteristic noninflammatory macrovascular and microvascular changes with dramatic and possibly occlusive formation of a thickened neointima. Scleroderma vessels also have an unusual endothelial phenotype, with loss of normal markers including vascular endothelial (VE)-cadherin. These endothelial cells express type 1 interferon and regulator of G protein signaling 5 (RGS5), two molecules associated with vascular rarefaction. These genes may be important because tissue is hypoxic with high levels of vascular endothelial growth factor (VEGF), especially early in the disease. The combination of VEGF and rarefaction is not necessarily paradoxical. VEGF-mediated angiogenesis creates labile vessels that may not survive unless the vessel acquires a smooth muscle coat. The combination of interferon and RGS5 is consistent with an antiangiogenic phenotype. We offer a hypothesis that places vascular injury at the center of this disease and also suggest possible clinical approaches for arresting and/or reversing the disease.

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“…The presence of vascular injury occurs in many tissues, including the skin, in the early phase of SSc (4). Vasospasm, characterized by Raynaud`s phenomenon, and a marked decrease in the number of capillaries in clinically involved and uninvolved skin has been reported, suggesting that endothelial cell death and defective angiogenesis might be responsible for the EC loss and vessel rarefaction in SSc (74). These pathological consequences lead to the presence of ischemic digital ulcers (DUs) with a marked decreased perfusion in the affected organs and the skin of SSc patients (75).…”

Section: Increased Ebv Dna Load Correlates With Clinical Patterns Of Vascular Injury In Ssc Patientsmentioning

confidence: 99%

Innate Immune Modulation Induced by EBV Lytic Infection Promotes Endothelial Cell Inflammation and Vascular Injury in Scleroderma

et al. 2021

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Microvascular injury is considered an initial event in the pathogenesis of scleroderma and endothelial cells are suspected of being the target of the autoimmune process seen in the disease. EBV has long been proposed as a trigger for autoimmune diseases, including scleroderma. Nevertheless, its contribution to the pathogenic process remains poorly understood. In this study, we report that EBV lytic antigens are detected in scleroderma dermal vessels, suggesting that endothelial cells might represent a target for EBV infection in scleroderma skin. We show that EBV DNA load is remarkably increased in peripheral blood, plasma and circulating monocytes from scleroderma patients compared to healthy EBV carriers, and that monocytes represent the prominent subsets of EBV-infected cells in scleroderma. Given that monocytes have the capacity to adhere to the endothelium, we then investigated whether monocyte-associated EBV could infect primary human endothelial cells. We demonstrated that endothelial cells are infectable by EBV, using human monocytes bound to recombinant EBV as a shuttle, even though cell-free virus failed to infect them. We show that EBV induces activation of TLR9 innate immune response and markers of vascular injury in infected endothelial cells and that up-regulation is associated with the expression of EBV lytic genes in infected cells. EBV innate immune modulation suggests a novel mechanism mediating inflammation, by which EBV triggers endothelial cell and vascular injury in scleroderma. In addition, our data point to up-regulation of EBV DNA loads as potential biomarker in developing vasculopathy in scleroderma. These findings provide the framework for the development of novel therapeutic interventions to shift the scleroderma treatment paradigm towards antiviral therapies.

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Capillary abnormalities, Raynaud's phenomenon, and systemic sclerosis in patients with localized scleroderma

Cited by 9 publications

References 0 publications

Capilaroscopia periungueal em crianças e adolescentes com doenças reumáticas

Capilaroscopia periungueal em crianças e adolescentes com doenças reumáticas

Is scleroderma a vasculopathy?

Innate Immune Modulation Induced by EBV Lytic Infection Promotes Endothelial Cell Inflammation and Vascular Injury in Scleroderma

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