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
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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.

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)

Prediction and primary prevention of major vascular complications (MVC) in systemic sclerosis (SSc). In SSc patients, our data confirm the role of arthritis and dyspnea as independent predictors of major vascular complications, in particular in MVCs-naïve patients. PubMed, Eur J Intern Med, 2021 Feb 5;S0953-6205(21)00022-4.

Does sublingual microscopy correlate with nailfold videocapillaroscopy in systemic sclerosis (SSc)? Nailfold and sublingual microscopy can identify a loss of capillary density in SSc patients. PubMed, Clin Rheumatol, 01/07/2021. (Also see Nailfold Capillaroscopy)

Neutrophil Extracellular Traps (NET) Generation Relates With Early Stage and Vascular Complications in Systemic Sclerosis (SSc). Neutrophils and especially NETosis represent new physiopathological and therapeutic fields in SSc. PubMed, J Clin Med, 2020 Jul 7;9(7):E2136.

Cardiovascular Risk Factors and Atherosclerotic Cardiovascular (CV) Events Among Incident Cases of Systemic Sclerosis (SSc). Patients with SSc are at ⦥2-fold increased risk for experiencing a CV event compared with persons without SSc. PubMed, Mayo Clin Proc, 2020 Jul;95(7):1369-1378.

Arterial stiffness correlates with progressive nailfold capillary microscopic changes in systemic sclerosis (SSc): results from a cross-sectional study. These data suggest that arterial stiffness correlates with microvascular damage in patients with SSc. PubMed, Arthritis Res Ther, 2019 Nov 27;21(1):253. (Also see Nailfold Capillaroscopy)

Association of simple hematological parameters with disease manifestations, activity, and severity in patients with systemic sclerosis (SSc). Globally available and inexpensive hematological tests, may be associated with vascular and cutaneous manifestations as well as disease activity and severity in SSc. PubMed, Clin Rheumatol, 07/18/2019.

Ulnar Artery Occlusion (UAO) and severity markers of vasculopathy in Systemic sclerosis (SSc): a multicenter cross–sectional study. UAO may represent a relevant severity marker of the vasculopathy in SSc. PubMed, Arthritis Rheumatol, 12/15/2018.

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)

Causes of Vascular Involvement in Scleroderma

CD21low B cells in systemic sclerosis: A possible marker of vascular complications. CD21low B cells are increased in SSc patients with visceral vascular manifestations. PubMed, Clin Immunol, 2020 Apr;213:108364. (Also see B Cells and T Cells)

Vascular biomarkers and digital ulcerations in systemic sclerosis: results from a randomized controlled trial of oral treprostinil (DISTOL-1). Longitudinal assessment of vascular biomarkers in a clinical trial setting provides a unique opportunity to define biomarkers that predict vascular outcomes. PubMed, Clin Rheumatol, 2020 Apr;39(4):1199-1205. (Also see 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.

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

Biomarker profiles of endothelial activation and dysfunction in rare systemic autoimmune diseases: implications for cardiovascular risk. Chronic inflammatory connective tissue diseases -specific biomarker profiles revealed an anti-angiogenic, interferon-driven environment during active disease. PubMed, Rheumatology (Oxford), 2020 Aug 18;keaa270.

Study on the relationship between hyperthyroidism and vascular endothelial cell damage. Hyperthyroidism can damage the vascular endothelium and is a high-risk factor for cardio-cerebrovascular disease. PubMed, Sci Rep, 2020 Apr 24;10(1):6992. (Also see Graves' Disease)

Prediction of organ involvement in systemic sclerosis (SSc) by serum biomarkers and peripheral endothelial function. This is the first study to inclusively investigate the relationships among biomarkers, noninvasive plethysmography-reactive hyperemia index and organ involvement in patients with SSc. PubMed, Clin Exp Rheumatol, Jul-Aug 2018;36 Suppl 113(4):102-108.

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, 2018 Feb;70(2):162-170. (Also see Digital Ulcers)

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.

Cryopreserved human adipose-derived stromal vascular fraction (SVF) maintains fracture healing potential via angiogenesis and osteogenesis in an immunodeficient rat model. SVF cells can enhance bone healing and cryopreserved cells have almost equal potential as fresh cells. PubMed, Stem Cell Res Ther, 2021 Feb 4;12(1):110.

Anti-endothelial cell antibodies (AECAs) are associated with apoptotic endothelial microparticles, endothelial sloughing and decrease in angiogenic progenitors in systemic sclerosis (SSc). We also identified a possible role of AECAs in the impairment of vascular repair in SSc as evidenced by significantly fewer angiogenic endothelial progenitor cells. PubMed, Postepy Dermatol Alergol, 2020 Oct;37(5):725-735.

Decreased CCN3 in Systemic Sclerosis endothelial cells contributes to impaired angiogenesis. CCN3 may represent a promising therapeutic target for SSc patients presenting with vascular involvement. PubMed, J Invest Dermatol, 01/16/2020.

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)

Significantly high concentrations of vascular endothelial growth factor (VEGF) in chronic subdural hematoma (CSDH) with trabecular formation. These results suggest that VEGF concentration in the hematoma alone could not be a reliable predictive biomarker for the natural history of CSDH including its recurrence. PubMed, Clin Neurol Neurosurg, 2021 Jan 6;202:106458.

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

Vitamin D in Vascular Calcification (VC): A Double-Edged Sword? This review addresses the current understanding of VC pathophysiology, with a focus on the pathogenic role of vitamin D that has provided new insights into the mechanisms of VC. PubMed, Nutrients. 2018 May 22;10(5). (Also see Vitamin D)

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.

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