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Vascular Involvement in Systemic Scleroderma

Author: Janey Willis. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer.
Vascular in Scleroderma
Types in Scleroderma
In Other CTDs
Personal Stories
Related Information

Overview of Vascular Disease

Abstract Painting  by Janet PaulmennThe 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.

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. (Also see: What is Scleroderma?, Raynaud's, and Watermelon Stomach)

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.
What Is Peripheral Arterial Disease? Peripheral arterial disease (P.A.D.) is a disease in which plaque builds up in the arteries that carry blood to your head, organs, and limbs. National Heart Lung and Blood Institute.
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)
Elevated pentraxin 3 in systemic sclerosis (SSc): Associations with vascular manifestations and defective vasculogenesis. In SSc patients, exposure to high PTX3 concentrations may promote vascular manifestations such as digital ulcers and pulmonary arterial hypertension. PubMed, Arthritis Rheumatol, 11/10/2014.
Safety, tolerability and potential efficacy of injection of autologous adipose-derived stromal vascular fraction (SVF) in the fingers of patients with systemic sclerosis: an open-label phase I trial. This study outlines the safety of the autologous SVF cells injection in the hands of patients with SSc and a significant improvement in hand disability and pain, Raynaud's phenomenon, finger oedema and quality of life was observed. PubMed, Ann Rheum Dis, 2014 Aug 11.
Arterial vasculopathy in systemic sclerosis (SSc): Computerized tomography (CT) angiographic features of macrovascular and microvascular upper limbs arteries. Macrovascular arterial vasculopathy of upper limbs may occur in SSc irrespective of the disease pattern and major arteries can be affected in association with other medium sized arteries of the forearms and microvascular arterial branches of the hands. PubMed, Joint Bone Spine, 2014 Jun 20.
A longitudinal study of ankle brachial pressure indices (ABPI) in a cohort of patients with systemic sclerosis (SSc). The severity of vascular disease in patients with SSc is associated with increasing age, smoking, limited cutaneous disease and positive anti-centromere antibodies. Reassuringly, in most patients ABPI remains stable over time. PubMed, Rheumatology (Oxford). 2014 Jun 6. (Also see: Digital Ulcers)
Correlations between nailfold microangiopathy severity, finger dermal thickness (DT) and fingertip blood perfusion (FBP) in systemic sclerosis patients (SSc). This study demonstrates a relationship between nailfold microangiopathy severity, DT and FBP in SSc patients. PubMed, Ann Rheum Dis, 2014 Jan 1;73(1):247-51.
Vasculitis patients at risk during times of stress. Dr. Robert Spiera, director of the Vasculitis and Scleroderma Program at the Hospital for Special Surgery, stated: "You should pay attention to the patient's mental well being and be more aggressive about intervening if a patient is in a bad place." Barchester. 07/11/2011.

Causes of Vascular Involvement in Scleroderma

How arguing with your partner could TREBLE your risk of dying young: Stress of squabbling can cause heart attacks and strokes. Individuals who argue and disagree for petty reasons are at an increased risk of dying young, according to a new study. Mail Online, 05/12/2014.
Severe vascular complications in patients affected by systemic sclerosis cyclically treated with iloprost. With the limits of an observational study and in the absence of a control group, our experience suggests that prolonged cyclic iloprost therapy may limit the incidence/progression of severe digital and visceral SSc-vasculopathy. Rheumatology International, 30 March 2011. (Also see: Iloprost)

Types of Vascular Involvement in Scleroderma

Brain Involvement. Vascular Headaches. ISN.
Progressive Loss of Lymphatic Vessels in Skin of Patients with Systemic Sclerosis (SSc). The progressive disappearance of lymphatic vessels may have a critical pathogenetic role in the progression of SSc from an early edematous phase to overt fibrosis. The Journal of Rheumatology. Nov 2010, 37 (11). (Also see: Skin Fibrosis)
Hearing loss in diffuse cutaneous systemic scleroderma (dcSSc). Patients with dcSSc have a high prevalence of sensorineural audiometric hearing impairment and otological complaints, suggesting that the cochlea is an additional target organ in this disease. Scandinavian Journal of Rheumatology, Sep 2011. (Also see: Ear 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.
Aortic Stiffness
Asymptomatic Pericardial
Cardiac Remodeling
Carotid Artery Disease
Left Ventricular Diastolic Dys
Lipoprotein[a], Thrombotic
Mitral/Tricuspid Valve
Myocardial Ischemia
Right Ventricular Dys
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?
A Patient's Perspective
Watermelon Stomach in Associated with Other Diseases
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. ISN.
Video Overview
Primary PH
Secondary PH
Exercised Induced PH
Disease Correlations
Research Registries
Patient Support
Personal Stories
Raynaud's Phenomenon. Raynaud's is a vascular disease commonly found in sclerodema. The fingers and/or toes become white and/or blue, and may become red on re-warming. ISN.
What is Raynaud's?
Improving Circulation
Rewarming Tips
Heatsock Directions
Tips from Patients
Patient Support
Patient Stories

Vascular Disease and Other Connective Tissue Diseases

Vascular manifestations of systemic lupus erythematosis. Awareness of the aetiology, pathophysiology, the clinical and histopathogical setting, and SLE-associated vascular complications is of great clinical significance. Neth J Med. 2013 Jan;71(1):10-6. (Also see: Lupus)

Biomarkers in Vascular Disease

Assessing microvascular changes in systemic sclerosis diagnosis and management. Microvascular damage and dysfunction represent the earliest morphological and functional markers of systemic sclerosis (SSc), a progressive connective tissue disease characterized by vascular abnormalities and diffuse fibrosis in the skin and internal organs. M Cutolo. (PubMed) Nat Rev Rheumatol. 2010 Aug 10. (Also see: Prognosis)

Angiogenesis and Vascular Disease

Understanding Angiogenesis. Abnormal blood vessel growth, either excessive or insufficient, is now recognized as a common denominator underlying many deadly and debilitating conditions, including cancer, skin diseases, age-related blindness, diabetic ulcers, cardiovascular disease, stroke, and many others. Angiogenesis Foundation.

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.

Vascular Endothelial Growth Factor (VEGF)

A vascular endothelial growth factor deficiency characterises scleroderma lung disease. Scleroderma interstitial lung disease is characterised by a VEGF deficiency. Lower concentrations were found in patients with progression of lung disease. Ann Rheum Dis 2012;71:1461-1465. (Also see: What is Scleroderma? and Pulmonary Involvement)

Personal Stories of Vascular Disease and Scleroderma

Raynaud's Personal Stories
Heart (Cardiac) Involvement Personal Stories
Pulmonary Hypertension Personal Stories
Watermelon Stomach (GAVE) Personal Stories

Related Information

Being Bullied Is Bad for Your Health. Being bullied raises the blood's level of C-Reactive Protein or CRP a marker of systemic inflammation and a risk factor for cardiovascular and other diseases. New York Times, 05/12/2014. (Also see: CRP: C-Reactive Protein)
As Temperatures Dip, Risk of Stroke Rises. Changes in temperature may impact stroke risk, hospitalization, and even death from stroke, new research says. Time Healthland, 02/12/2014.
IgG4-Associated Vasculitis. Clinicians must be aware that the clinical manifestations of IgG4-RD and anti-neutrophil cytoplasmatic antibodies (ANCA)-associated vasculitis may overlap. PubMed. Curr Rheumatol Rep, 2013 Aug;15(8):348.
Extra fiber tied to lower risk of stroke. People who get more fiber in their diet are less likely to have a stroke than those who skimp on the nutrient, according to a new review of existing research. Chicago Tribune. 04/19/2013. (Also see: Food Guide for Autoimmune Disease: Fiber)
Vascular Smooth Muscle Cells (SMC) Progenitors: New Players In Systemic Sclerosis (Ssc) Defective Vessel Regeneration. The ability of MSC to improve the endothelial cells (ECs) suggest they could be candidates for regenerative medicine during SSc. P. Cipriani. EULAR 2011 OPO 240. Ann Rheum Dis 2011;70(Suppl3):150.
Vascular Disease Foundation. 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. Vascular Disease Foundation.
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