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Vascular Involvement in Systemic Scleroderma

Author: Janey Willis. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer.
Overview
Vascular in Scleroderma
Causes
Types in Scleroderma
In Other CTDs
Biomarkers
Angiogenesis
Hypoxia
VEGF
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.

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)

Correlations between blood perfusion (BP) and dermal thickness (DT) in different skin areas of systemic sclerosis (SSc) patients. This study demonstrates for the first time in SSc patients a significant inverse relationship between skin BP, measured by laser speckle contrast analysis, and DT, evaluated by both ultrasound and modified Rodnan skin score (mRSS), at the level of dorsum of the middle phalanx of the third fingers. PubMed, Microvasc Res, 2018 Jan;115:28-33. (Also see Skin Fibrosis)

Prevalence of macrovascular arterial involvement of the 4 limbs in systemic sclerosis (SSc): About a case series of 14 patients. It seems necessary that SSc patients have a strict balance of their cardiovascular risk factors (CVRF) and a screening of macrovascular arterial lesions. PubMed, Rev Med Interne. 2017 Jul;38(7):430-435.

Exercise–induced brachial artery blood flow and vascular function is impaired in systemic sclerosis (SSc). Vascular dysfunction in the patients with SSc was accompanied by elevated blood markers of oxidative stress and attenuated endogenous antioxidant activity. PubMed, Am J Physiol Heart Circ Physiol, 2016 Dec 1;311(6):H1375-H1381.

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)

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, Arthritis Rheumatol, 2015 Feb;67(2):498-507.

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.

Causes of Vascular Involvement in Scleroderma

Risk of Digital Vascular Events in Scleroderma Patients Who Have Both Anticentromere and Anti-Interferon-Inducible Protein 16 Antibodies. Scleroderma patients who are double–positive for antibodies recognizing CENP and IFI-16 are significantly more likely to have significant digital vascular events during the course of their disease. PubMed, Arthritis Care Res (Hoboken), 2017 Jun;69(6):922-926. (Also see Antibodies and Digital Ulcers)

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.

Overview
Antiphospholipid
Aortic Stiffness
Asymptomatic Pericardial
Cardiac Remodeling
Carotid Artery Disease
Cholesterol
Atherosclerosis
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?
Diagnosis
Treatment
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
Causes
Disease Correlations
Symptoms
Diagnosis
Prognosis
Treatments
Research Registries
Research
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.

Overview
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

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, 2015 Apr;14(4):314-22.

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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