Carotid Artery Disease
Stroke and TIA
The systemic forms of scleroderma can cause cardiac (heart) problems. One of the earliest symptoms reported by diffuse systemic scleroderma patients is heart irregularities.
Systemic scleroderma can cause arrhythmias, pericardial effusion, and heart failure. Formation of scar tissue in the heart and lungs has recently been recognized as a general pattern probably contributing to overall fatigue. (Also see What is Scleroderma?, Types of Scleroderma, Symptoms of Scleroderma)
Heart involvement in systemic sclerosis. Primary and secondary heart involvement in systemic sclerosis are important mortality predictors and left ventricular diastolic impairment was the most frequent echographic abnormality in our study. PubMed, Tunis Med. 2017 Mar;95(3):215-220.
Cardiac involvement (CI) in systemic sclerosis (SSc) : differences between clinical subsets and influence on survival. CI increased the mortality and appeared at a higher rate, especially during the first year after SSc onset and screening for heart involvement should be performed at diagnosis and during follow-up. PubMed, Rheumatol Int, 2017 Jan;37(1):75-84.
The importance of fragmented QRS (fQRS) in the early detection of cardiac involvement in patients with systemic sclerosis (SSc). The presence of fQRS in SSc patients, were more frequent than in the normal population. PubMed, Anadolu Kardiyol Derg, 2015 Mar;15(3):209-12.
Cardiomyopathy in murine models of systemic sclerosis (SSc). Fra-2 tg mice might be a suitable preclinical model to study the mechanisms and therapeutic approaches of myocardial involvement in SSc. PubMed, Arthritis Rheumatol, 2015 Feb;67(2):508-16.
Cardiac biomarkers in systemic sclerosis: contribution of high-sensitivity cardiac troponin in addition to N-terminal pro-brain natriuretic peptide. The easily obtained biomarkers may be useful for systematic evaluation and stratification of SSc patients, especially to identify those at risk of pulmonary hypertension. PubMed, Arthritis Care Res (Hoboken), 01/20/2015. (Also see Scleroderma Biomarkers)
Antiphospholipid Antibodies in Systemic Sclerosis. Antiphospholid antibodies are frequently found in systemic scleroderma patients. They are correlated with pulmonary hypertension in scleroderma. ISN.
APS and Heart Disease
Scleroderma and APS
Causes and Research
Aortic Stiffness is a loss of elasticity in the aortic wall. Aortic stiffness is an important prognosticators of cardiovascular disease risk.
Aortic pulse wave velocity (aPWV) measurement in systemic sclerosis (SSc) patients. A significantly higher prevalence of abnormally increased aPWV was evidenced in SSc patients compared to healthy controls. PubMed, Reumatismo.
What is Atherosclerosis? Atherosclerosis, often described as a hardening of the arteries, occurs when the normal lining of the arteries deteriorates, the walls of arteries thicken, and deposits of fat and plaque build up, causing narrowing (or even blockage) of the arteries. WebMD.
Atherosclerosis and autoimmunity: a growing relationship. Being armed with the mechanisms underlying autoimmunity in the etiopathogenesis of atherosclerosis in rheumatic autoimmune disorders and the shared etiologic pathway may result in substantial developing therapeutics for these patients. PubMed, Int J Rheum Dis, 04/19/2018.
Vitamin D deficiency is associated with digital ulcer (DU) but not with atherosclerosis or arterial stiffness in patients with systemic sclerosis: a pilot study. Vitamin D deficiency was associated with DUs, but not with atherosclerosis or arterial stiffness, potentially indicating that vitamin D may have different effects on the microvascular and macrovascular involvement in SSc pathophysiology. PubMed, Clin Rheumatol, 2017 Jun;36(6):1325-1333. (Also see Digital Ulcers and Vitamin D)
Early morphologic and functional changes of atherosclerosis in systemic sclerosis-a systematic review and meta–analysis. Meta–analysis of the published observational studies confirms that abnormalities attributable to macrovascular involvement are significantly more prevalent in SSc patients compared with controls. PubMed, Rheumatology (Oxford), 08/25/2016.
A critical view on cardiovascular risk in systemic sclerosis. We review the surrogate markers of CVD that have been evaluated and examine whether common pathogenic mechanisms exist between SSc and macrovascular disease. PubMed, Rheumatol Int, 07/12/2016.
Systemic Sclerosis (SSc) Patients Are More Prone to Atherosclerosis. Patients with SSc display an increased risk for subclinical atherosclerosis and peripheral arterial disease when compared to healthy individuals. Scleroderma News, 04/17/2015.
Asymptomatic means without symptoms. Pericardial means heart, and the area around the heart. Scleroderma can cause asymptomatic heart disease.
Silent cardiovascular involvement in patients with diffuse scleroderma: A controlled cross-sectional study. This study showed that those SSc patients without any clinical evidence of CVD seem to have subclinical atherosclerosis, which was suggested by early impairment of coronary microcirculation and macrovascular involvement. Wiley. Arthritis Care & Research.
The majority of patients with systemic scleroderma are believed to have subclinical primary cardiac involvement. Symptoms may manifest without warning and can rapidly lead to arrhythmia and left and right heart dysfunction and failure. Oxford, Rheumatology (expired article).
A qualitative systematic review of the prevalence of coronary artery disease (CAD) in systemic sclerosis (SSc). SSc is an independent risk factor and is associated with an increased prevalence/incidence for CAD. PubMed, Int J Rheum Dis, 2015 Mar;18(3):276-86.
Prevalence of subclinical atherosclerosis is increased in systemic sclerosis and is associated with serum proteins: a cross-sectional, controlled study of carotid ultrasound. Patients with SSc have a higher prevalence of carotid plaque than matched controls. PubMed, Rheumatology (Oxford).
On average, systemic scleroderma patients do not differ from healthy people, in terms of total body fat, despite the ravages of illness and frequent complications of gastrointestinal difficulties. Another unusual finding in the following study is that scleroderma patients had significantly better cholesterol profiles than healthy people. (Also see Systemic Scleroderma)
Cholesterol–Slashing Drug Can Protect High–Risk Heart Patients, Study Finds. The first rigorous test of an expensive new drug that radically lowers cholesterol levels found that it significantly reduced the chance that a high–risk patient would have a heart attack or stroke. New York Times, 03/17/2017.
Case Report: Autoimmune myocarditis in systemic sclerosis: an unusual form of scleroderma heart disease presentation. Screening and early diagnosis of cardiac involvement is important, because a timely treatment may impact the quality of life of these patients and improve their prognosis. PubMed, ESC Heart Fail. 2017 Aug;4(3):365-370.
Incremental Value of Two Dimensional Speckle Tracking Echocardiography in the Functional Assessment and Characterization of Subclinical Left Ventricular Dysfunction. This article highlights the incremental utility of 2D-STE in the detection of subclinical LV dysfunction. PubMed, Curr Cardiol Rev. 2017;13(1):32-40.
What Is Diastolic Dysfunction and Diastolic Heart Failure? An abnormal "stiffening" of the ventricles, and the resulting abnormal ventricular filling during diastole, is referred to as diastolic dysfunction. VeryWell.
Diastolic Dysfunction can also be a complication of systemic scleroderma. ISN
Thrombotic complications after radial arterial line placement in systemic sclerosis: A case series. Radial arterial line placement may trigger critical ischemic events in scleroderma patients which suggests that placement of radial lines needs to be thoughtfully weighed prior to insertion. Seminars in Arthritis and Rheumatism,04/02/2016.
Tricuspid Regurgitation. Tricuspid regurgitation (tricuspid incompetence, tricuspid insufficiency) is leakage of blood backward through the tricuspid valve each time the right ventricle contracts. Merck.
Cardiac (Myocardial) ischemia. Cardiac ischemia occurs when blood flow to the heart muscle (myocardium) is obstructed by a partial or complete blockage of a coronary artery. Mayo Clinic.
Noninvasive Contrast-Enhanced Ultrasound Molecular Imaging Detects Myocardial Inflammatory Response in Autoimmune Myocarditis. Contrast–enhanced ultrasound molecular imaging can detect endothelial inflammation and leukocyte infiltration in myocarditis and imaging of CD4+ T cells involved in autoimmune responses could be helpful in diagnosing it. PubMed, Circ Cardiovasc Imaging, 2016 Aug;9(8).
Mechanics and prognostic value of left (LV) and right ventricular (RV) dysfunction in patients with systemic sclerosis. RV dysfunction was associated with adverse outcome, independent of and incremental to clinical and LV deformation parameters in SSc. European Heart Journal, 08/11/2017.
Cardiopulmonary exercise testing (CPET) for detecting pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc). CPET is a safe and valuable method in the non-invasive detection of SSc–associated PAH and it may be particularly beneficial for reducing unnecessary right heart catheterisation procedures. PubMed, Heart, 01/06/2017. (Also see Secondary Pulmonary Hypertension)
Impact of pulmonary fibrosis (PF) and elevated pulmonary pressures (PHT) on right ventricular (RV) function in patients with systemic sclerosis (SSc). SSc patients show impaired RV function and both pulmonary fibrosis and PHT are independently associated with RV dysfunction. PubMed, Rheumatology (Oxford),2016 Mar;55(3):504-12. (Also see Pulmonary Hypertension)
A Right Ventricular (RV) Diastolic Impairment is Common in Systemic Sclerosis (SSc) and is Associated with Other Target-Organ Damage. RV is commonly affected in SSc with predominant impaired diastolic function and several factors, including primary heart, lung vascular disease and pulmonary hypertension, contribute to such impairment. Seminars in Arthritis and Rheumatism, 07/07/2015.
What is a Stroke? Stroke is the third leading cause of death in America and the No. 1 cause of adult disability. 80% of strokes are preventable; you can prevent a stroke! Someone who has a small stroke may experience only minor problems such as weakness of an arm or leg. People who have larger strokes may be paralyzed on one side or lose their ability to speak. National Stroke Association.
What is a Transient Ischemic Attack (TIA)? It is a stroke that lasts only a few minutes. Symptoms can include numbness or weakness in the face, arm, or leg, especially on one side of the body; confusion or difficulty in talking or understanding speech; trouble seeing in one or both eyes; and difficulty with walking, dizziness, or loss of balance and coordination. National Institute of Neurological Disorders and Stroke.
Reproductive Factors in Women Tied to Heart Disease and Stroke Risk. Several female reproductive factors are associated with an increased risk for heart disease and stroke, British researchers report. New York Times, 01/18/2018.
Stroke survivors without complications still face ongoing risks. Even stroke survivors who don’t experience any immediate complications are still more likely to die or have a heart attack than people who never had a stroke, a recent Canadian study suggests. Reuters Health, 07/25/2017.
Diet Sodas Raise Risk of Dementia and Stroke, Study Finds. People who drink diet sodas daily have three times the risk of stroke and dementia compared to people who rarely drink them, researchers reported. NBC Health News, 04/20/2017.
Marital status, history linked to survival after stroke. A person’s current marital status and past marital losses may be related to their survival odds after a stroke, a new study suggests. Reuters Health, 12/14/2016.
Stroke survivors may face invisible obstacles returning to work. Stroke survivors may face memory, concentration and fatigue problems when returning to work, according to an analysis of a U.K. online forum. Reuters Health, 04/13/2016.
Endocannabinoids and the Cardiovascular System in Health and Disease. In neurovascular disorders such as stroke, endocannabinoids are upregulated and protective. Although most of this evidence is from preclinical studies, it seems likely that cannabinoid–based therapies could be beneficial in a range of cardiovascular disorders. PubMed, Handb Exp Pharmacol, 2015;231:393-422. (Also see Endocannabinoid System)
Ischemic stroke as the first manifestation of granulomatosis with polyangiitis. Granulomatosis with polyangiitis should be considered in the differential diagnosis of ischemic stroke even in the absence of extraneurological involvement. PubMed, Rev Med Interne. (Also see Granulomatosis with Polyangiitis)
Patient and Caregiver Stories about Scleroderma Heart Involvement.
Barb: Diffuse Systemic Scleroderma I was diagnosed in 1985 with systemic scleroderma and Raynaud's. It has affected my esophagus, fingers, toes and bowels, and recently I started having mini strokes…
Papa V: Lupus, Antiphospholipid Syndrome, Myasthenia Gravis, and Scleroderma About thirty years passed, and then—BAM !—I got a heart blockage, stroke, liver biopsy due to elevated enzymes, skin rash, high blood pressure, very high ANA…
Read more Patient and Caregiver Stories about Scleroderma Heart Involvement.
Many thanks to our recent donors, Jack and Elizabeth Lewis, Chris Dokter, Margaret Roof, Network for Good, PayPal Charitable Giving Fund, United Way Snohomish and Arnold Slotkin. See ISN News for more donors. Click Here to Donate.
TOLL FREE HOTLINE, U.S. and Canada: 1-800-564-7099
Ask for our Free Info Packet by email or postal mail!
SCLERO.ORG is the world's leading nonprofit for trustworthy research, support, education and awareness for scleroderma and related illnesses.
We are a service of the nonprofit International Scleroderma Network (ISN), which is a 501(c)(3) U.S.-based public charitable foundation, established in 2002. Meet Our Team, or Volunteer. Donations may also be mailed to: