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Vascular Involvement in Systemic Scleroderma
Janey Willis Hello, I am Janey Willis, ISN Guide to Vascular Involvement. Systemic sclerosis (scleroderma) can affect any or every part of the body, including the circulatory system (vascular system). Problems in the circulatory system can cause heart problems, Raynaud's, Watermelon Stomach, and even vascular headaches. This page has not yet been medically edited. Please keep in mind that just because something is listed here, it does not mean an individual patient will ever experience it. See Disclaimer.
Overview of Vascular Disease
Vascular Involvement in Scleroderma
Causes of Vascular Involvement
Types of Vascular Disease in Scleroderma
Vascular Disease in Other Connective Tissue Disease
Biomarkers in Vascular Disease

Angiogenesis and Vascular Disease
Hypoxia and Vascular Disease
Vascular Endothelial Growth Factor (VEGF)
Personal Stories

Related Information
Overview of Vascular Disease
The vascular system is our circulatory system. It consists of all the vessels that carry blood and lymph through the body, to and from organs. Vessels include arteries, veins, and capillaries. Any condition that affects the vascular system, all of in part, is considered a vascular disease.
The Heart and Vascular Disease. Vascular disease includes any condition that affects the circulatory system. Vascular disease ranges from diseases of your arteries, veins, and lymph vessels to blood disorders that affect circulation. WebMD.
Peripheral Vascular Disease. This refers to diseases of blood vessels outside the heart and brain. It's often a narrowing of vessels that carry blood to the legs, arms, stomach or kidneys. American Heart Association.
Disease Information about Types of Vascular Diseases. Vascular disease includes Raynaud's Disease and Phenomenon, Peripheral Arterial Disease, Deep Vein Thrombosis, Stroke, and Varicose Veins.
Vascular Involvement in Systemic Scleroderma
Vascular disease in scleroderma can be widespread throughout the body. In effect, vascular disease can affect any organ or any part of the body by affecting the circulation of blood to and from or within. Examples are Raynaud's Phenomenon, Pulmonary Arterial Hypertension, and Renal Involvement. (Also see: What is Scleroderma? and Types of Scleroderma)
Vascular disease in scleroderma. Although scleroderma is generally considered a fibrosing disease of the tissues, it is now recognized that the underlying vascular disease is playing a fundamental role in its pathogenesis and associated tissue injury. Wigley FM. (PubMed) Clin Rev Allergy Immunol. 2009 Jun;36(2-3):150-75.
Long-term beneficial effects of statins on vascular manifestations in patients with systemic sclerosis. This pilot study suggests that statins may be beneficial in treating vascular manifestations of SSc, such as Raynaud’s, through their pleiotropic effects. However, this treatment did not correct the defect in endothelial progenitor cells recruitment. Masataka Kuwana. (SpringerLink) Modern Rheumatology July 10, 2009. (Also see: Statins and Raynaud's)
Improved endothelial function after endothelin (ET(A)) receptor blockade in patients with systemic sclerosis (SSc). The results of this study suggest novel vasculoprotective effects of ET(A) receptor antagonism and support further exploration of strategies that target the endothelin 1 pathway in SSc. C. Cardillo. (PubMed) Arthritis Rheum. June 2009 (Also see: Endothelin)
Reduced exercise capacity in systemic sclerosis (SSc) patients without pulmonary involvement. SSc patients without pulmonary impairment have reduced exercise capacity. Abnormal vascular response to exercise may account for this finding, as the vascular system is one of the major target organs in this pathological condition. Scandinavian Journal of Rheumatology, Volume 36, Issue 6 2007, pages 458 - 461. (InformaWorld) (Also see: Fatigue)
Is scleroderma a vasculopathy? 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. Fleming JN. (PubMed) Curr Rheumatol Rep. 2009 Apr;11(2):103-10.
Causes of Vascular Involvement in Scleroderma
Reduced Aerobic Capacity in Systemic Sclerosis (SSc) Patients Without Pulmonary Involvement. Abnormal vascular response to exercise may account for this finding since the vascular system is one of the major target organs in this pathologic condition. Natalia C. Oliveira. 1866/538 ACR 2006.
Bone marrow endothelial progenitors are defective in systemic sclerosis (SSc). This study showed there is a complex impairment in the bone marrow microenvironment involving both the endothelial and mesenchymal stem cell compartments and that this impairment might play a role in defective vasculogenesis in scleroderma. PubMed. Arthritis Rheum. 2006 Jul 25;54(8):2605-2615.
Hyperplasia of dermal microvascular pericytes (PC) in scleroderma. The observed pericytes increase in the peripheral zones of active disease supports the hypothesis of a vascular pathogenesis of scleroderma and directs the focus to microvascular PC. PubMed. J Cutan Pathol. 2004 Jul;31(6):431-40.
Defective vasculogenesis in systemic sclerosis. Insufficient vascular repair machinery due to defective vasculogenesis might contribute to vasculopathy in systemic sclerosis. Circulating endothelial precursors could be a novel target for therapeutic strategies for ischaemic complications in patients with systemic sclerosis. PubMed. Lancet. 2004 Aug 14;364(9434):603-10.
Types of Vascular Involvement in Scleroderma
Brain Involvement. Vascular Headaches. ISN.
Cerebral Vasculopathy Is Associated with Severe Vascular Manifestations in Systemic Sclerosis. Severe cerebral vasculopathy is associated with severe vascular manifestations in SSc patients. SSc patients with severe vascular complications should undergo neuroradiological imaging assessment of brain involvement. Benjamin Terrier. The Journal of Rheumatology. vol. 36 no. 7 1486-1494. July 2009. (Also see: Brain Involvement)
Systemic Scleroderma Cardiac (Heart) Involvement. Vascular disease and the heart involves the blood vessels to and from the heart. Carotid artery disease, atherosclerosis, and stroke are types of vascular disease that can affect the heart. ISN.
Overview of SD Cardiac Involvement
Antiphospholipid Antibodies
Aortic Stiffness
Asymptomatic Pericardial Disease
Cardiac Remodeling
Carotid Artery Disease
Cholesterol
Atherosclerosis
Left Ventricular Diastolic Dysfunction
Lipoprotein[a], Thrombotic
Mitral and Tricuspid Valve
Myocardial Ischemia
Right Ventricular Dysfunction
Stroke and TIA
Gastric Antral Vascular Ectasia (GAVE). Watermelon Stomach is the popular name for Gastric Antral Vascular Ectasia (GAVE) — a condition in which the lining of the stomach bleeds, causing it to look like the characteristic stripes of a watermelon when viewed by Endoscopy. ISN
What is Watermelon Stomach?
Diagnosis
Treatment
A Patient's Perspective
Research
Patient/Caregiver Stories
Pulmonary Arterial Hypertension. Pulmonary Hypertension is not the same as "regular" high blood pressure or hypertension, which can be easily measured with a blood pressure test. Rather, it is a very difficult to detect elevation in pressure in specific arteries which take blood between the heart and the lungs. Pulmonary Hypertension is a type of vascular disease that can be found in scleroderma patients. ISN.
What is PH?
Primary PH
Isolated PH
Secondary PH
Exercised Induced PH
Causes of PH

PH Disease Correlations
Symptoms
Diagnosis
Prognosis

Treatments
Research Registries
Research
Patient Support Organizations
Personal Stories
Raynaud's Phenomenon. Raynaud's is a vascular disease commonly found in sclerodema. It is an extreme spasm of blood vessels in response to cold or stress. The fingers and/or toes become white and/or blue, and may become red on re-warming. ISN.
What is Raynaud's?
Diagnosis
Treatments
Medications
Improving Circulation

Prevention
Rewarming Tips
Heatsock Directions

Tips from Patients
Research
Patient Support

Patient Stories
Vascular Disease and Other Connective Tissue Diseases
Prevalence, predictors and outcome of vascular damage in systemic lupus erythematosus (SLE). Vascular events (VE) occurred in 26% of SLE patients, predominantly as atherothrombotic disease. VE prevalence increased linearly over time leading to a four-fold risk of mortality. (SageJournals). A. Becker-Merok. Lupus. April 2009. (Also see: Lupus)
Biomarkers in Vascular Disease
Biomarkers of vascular disease in scleroderma. Vascular disease is present in every patient with scleroderma and is a major source of morbidity and mortality. There is a subset of patients who will develop severe and sometimes life-threatening vascular events. Hummers LK. (PubMed) Rheumatology (Oxford). 2008 Oct;47 Suppl 5:v21-2.
Angiogenesis and Vascular Disease
Angiogenesis. Angiogenesis is a natural process in the body that involves the growth of new blood vessels. It can occur during coronary artery disease, peripheral artery disease and stroke when there's insufficient blood supply and oxygen to the tissues, a condition known as ischemia. American Heart Association.
Abnormalities in the Regulators of Angiogenesis in Patients with Scleroderma. The levels of some factors correlate with measures of vascular disease among patients with scleroderma. Dysregulated angiogenesis may play a role in the development of scleroderma vascular disease. L. Hummers. The Journal of Rheumatology March 1, 2009 vol. 36 no. 3 576-582.
Hypoxia and Vascular Disease
Hypoxia (Medical). Hypoxia is a pathological condition in which the body as a whole (generalized hypoxia) or a region of the body (tissue hypoxia) is deprived of adequate oxygen supply. Wikipedia.
Effects of Iron Supplementation and Depletion on Hypoxic Pulmonary Hypertension Two Randomized Controlled Trials. Hypoxia is a major cause of pulmonary hypertension in respiratory disease and at high altitude. Hypoxic pulmonary hypertension may be attenuated by iron supplementation and exacerbated by iron depletion. Thomas G. Smith, MBBS, DPhil. JAMA. 2009;302(13):1444-1450. (Also see: Pulmonary Hypertension and Vitamins/Minerals)
Hypoxia. Hypoxia in the pathogenesis of systemic sclerosis. Severe hypoxia is a characteristic feature of SSc and might contribute directly to the progression of the disease by augmenting vascular disease and tissue fibrosis. Christian Beyer. Arthritis Research & Therapy. 21 April 2009. 11:220.
Vascular Endothelial Growth Factor (VEGF)
The transport of high amounts of vascular endothelial growth factor (VEGF) by blood platelets underlines their potential contribution in systemic sclerosis angiogenesis. Platelets transport high levels of VEGF in SSc. They may contribute to circulating VEGF because of ongoing activation in the course of the disease. If activated at the contact of injured endothelium, platelets may be important in the altered angiogenesis associated with the disease through the secretion of high levels of VEGF. Anne Solanilla. Rheumatology June 23, 2009.
Serum Vascular Endothelial Growth Factor (VEGF) levels are related to the presence of pulmonary arterial hypertension (PAH) in systemic sclerosis. Serum VEGF levels are increased in systemic sclerosis patients with systolic pulmonary artery pressure(sPAP) ≥ 35 mmHg. The correlation between VEGF levels and sPAP may suggest a possible role of VEGF in the pathogenesis of PAH in systemic sclerosis. Andriana I Papaioannou. BMC Pulmonary Medicine 2009, 9:18. (Also see: Pulmonary Hypertension)
Personal Stories: Vascular Disease and Scleroderma
Raynaud's Personal Stories
Heart (Cardiac) Involvement Personal Stories
Pulmonary Hypertension Personal Stories
Watermelon Stomach (GAVE) Personal Stories
Related Information
Vascular Disease Foundation (VDF). Peripheral vascular diseases are recognized by the general public and by the health care community as disabling and deadly for which the quality of life and the longevity of affected individuals can be improved through increased education, prevention, prompt diagnosis, comprehensive treatment and effective rehabilitation. The efforts of VDF will result in reduced disability and death from vascular disease. Vascular Disease Foundation.
 
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