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
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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.

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 stiffness mechanoactivates YAP/TAZ–dependent glutaminolysis to drive pulmonary hypertension. These results indicate that extracellular matrix stiffening sustains vascular cell growth and migration through YAP/TAZ-dependent glutaminolysis and anaplerosis, and thereby link mechanical stimuli to dysregulated vascular metabolism. PubMed, J Clin Invest, 2016 Sep 1;126(9):3313-35.

Autoantigens targeted in scleroderma patients with vascular disease are enriched in endothelial lineage cells. Expression of scleroderma autoantigens IFI16 and CENPs, which are associated with severe vascular disease, is increased in vascular progenitors and mature endothelial cells. PubMed, 05/09/2016.

Autoantigens targeted in scleroderma patients with vascular disease are enriched in endothelial lineage cells. Expression of scleroderma autoantigens IFI16 and CENPs, which are associated with severe vascular disease, is increased in vascular progenitors and mature endothelial cells. PubMed, Arthritis Rheumatol, 05/09/2016.

Consensus best practice pathway of the UK Systemic Sclerosis Study Group: digital vasculopathy in systemic sclerosis. A number of treatment algorithms are provided that are intended to provide the clinician with accessible reference tools for use in daily management. PubMed, Rheumatology (Oxford), 06/26/2015.

The relationship between nailfold capillaroscopic assessment and telangiectasia score with severity of peripheral vascular involvement in systemic sclerosis. Digital ulcer history and severe PVI in SSc were associated with capillary loss and microangiopathy. PubMed, Clinical and Experimental Rheumatology, 03/23/2015. (Also see Nailfold Videocapillaroscopy and Telangiectasia)

Decreased numbers of endothelial progenitor cells (EPCs) in patients in the early stages of systemic sclerosis (SSc). Decreased EPCs in SSc patients might be an important therapeutic target for the prevention of vascular complications. PubMed, Microvasc Res, 2015 Jan 13;98C:82-87.

Systemic sclerosis (SSc) induces pronounced peripheral vascular dysfunction characterized by blunted peripheral vasoreactivity and endothelial dysfunction. Compared to healthy controls, SSc patients had significantly smaller brachial artery diameter and blunted peripheral vascular reactivity and endothelial function. PubMed, Clin Rheumatol, 12/16/2014.

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, 06/20/2014.

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). 06/06/2014. (Also see Digital Ulcers)

Vascular events are risk factors for anal incontinence (AI) in systemic sclerosis: a study of morphology and functional properties measured by anal endosonography and manometry. SSc patients with AI have a thin internal anal sphincter and a low resting pressure. Risk factors for AI among SSc patients are centromeric antibodies and vascular disease. PubMed, Scand J Rheumatol. 2014;43(5):391-7. (Also see Bowel Dysfunction in Systemic Scleroderma)

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.

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.

Types of Vascular Involvement in Scleroderma

Brain Involvement. Vascular Headaches. ISN.

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 Ventricle
Lipoprotein[a], Thrombotic
Mitral/Tricuspid Valve
Myocardial Ischemia
Right Ventricle
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.

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. (Also see Lupus)

Biomarkers in Vascular Disease

Defining a unified vascular phenotype in systemic sclerosis. The more recent biomarker longitudinal studies support the concept of generalized vasculopathy and a potential association between various cardiovascular complications. PubMed, Arthritis Rheumatol, 11/16/2017. (Also see Digital Ulcers)

Serum levels of genomic DNA of α1(I) collagen are elevated in scleroderma patients. In summary, elevation of serum α1(I) collagen DNA levels in scleroderma patients may be useful as the diagnostic marker, reflecting the presence of vasculopathy. PubMed, J Dermatol, 03/31/2017.

Vascular Biomarkers and Correlation with Peripheral Vasculopathy in Systemic Sclerosis. Vascular biomarkers could become useful predictive factors of vascular damage in SSc, allowing an earlier management of vascular complications. PubMed, Autoimmun Rev, 12/04/2014.

Angiogenesis and Vascular Disease

Angiogenesis. A tumor consists of cells that are dividing at an abnormally high rate, crowding surrounding healthy cells and competing for resources. BioInteractive.

The "myth" of loss of angiogenesis in systemic sclerosis: a pivotal early pathogenetic process or just a late unavoidable event? We suggest the loss of angiogenesis should not be considered as an overall "myth" characterizing systemic sclerosis, but as a very late event of the vascular pathogenesis. PubMed, Arthritis Res Ther, 2017 Jul 6;19(1):162.

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)

The role of endothelial cells (EC) in the vasculopathy of systemic sclerosis (SSc): A systematic review. This systematic review emphasizes the growing evidence that SSc is primarily a vascular disease where EC dysfunction is present and prominent in different aspects of cell survival. PubMed, Autoimmun Rev, 05/29/2017.

Nailfold videocapillaroscopy (NVC) and serum VEGF levels in scleroderma (SSc) are associated with internal organ involvement. Our data suggest the importance of NVC for the diagnosis of SSc patients and serum VEGF levels may act as a biomarker of interstitial lung involvement. PubMed, Auto Immun Highlights. 2016 Dec;7(1):5.

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

Help brains by cooling bodies after cardiac arrest. People who are left unconscious after their heart suddenly stops should be cooled in an effort to preserve brain function, according to the American Academy of Neurology. Reuters Health, 05/12/2017.

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.

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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