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Systemic Scleroderma Cardiac (Heart) Involvement

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
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

Overview

Abstract paintingThe 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)

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)

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. ResearchGate Arthritis & Rheumatology, 10/2014.

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, 04/08/2014.

Systemic sclerosis and the heart. Although no specific therapy exists for scleroderma heart disease, early recognition of the presence and type of scleroderma heart disease may lead to more effective management of patients. PubMed, Rheum Dis Clin North Am, 2014 Feb;40(1):87-102.

Abnormal cardiac enzymes in systemic sclerosis. Cardiac biomarkers such as troponin T (TnT) may be useful screening tools to identify subclinical cardiac disease and assess response to therapeutic intervention. Clinical Rheumatology, October 2013.

Relationship between interleukin-6 (IL-6) and cardiac involvement in systemic sclerosis (SSc). These results support the role of IL-6 in the development of cardiac disease in SSc patients. PubMed, Rheumatology, 03/27/2013. (Also see Causes of Scleroderma: Interleukins)

Antiphospholipid Antibodies in Scleroderma (Systemic Sclerosis)

Antiphospholipid Antibodies in Systemic Sclerosis. Antiphospholid antibodies are frequently found in systemic scleroderma patients. They are correlated with pulmonary hypertension in scleroderma. ISN.

Overview
APS and Heart Disease
Scleroderma and APS
Diagnosis
Symptoms
Complications
Treatments
Causes and Research
Support Resources
Personal Stories

Aortic Stiffness

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, 2012 Dec 20;64(6).

Atherosclerosis

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.

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.

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.

Is atherosclerosis accelerated in systemic sclerosis (SSc)? Novel insights. Although microvascular disease is a hallmark of SSc, mechanistic insights explaining the presence of accelerated atherosclerosis, and the presence and extent of macrovascular disease in SSc patients are lacking and future challenges will be to unravel the genetic, environmental and ethnic determinants of such processes. PubMed, Curr Opin Rheumatol, 2014 Nov;26(6):653-7.

No association of atherosclerosis with digital ulcers (DU) in Japanese patients with systemic sclerosis (SSc): evaluation of carotid intima-media thickness (IMT) and plaque characteristics. There were no differences in serum lipid level, carotid IMT or plaque score between SSc patients with and without DU, suggesting that atherosclerotic changes are not primarily involved in the development of DU. PubMed, J Dermatology, 2014 Jul;41(7):604-8. (Also see Digital Ulcers)

Subclinical Atherosclerosis Noted in Diffuse Scleroderma. Patients with diffuse systemic sclerosis (SSc), without any clinical evidence of cardiovascular disease, have indicators of subclinical atherosclerosis. Doctors Lounge, 08/29/12.

Asymptomatic Pericardial Disease

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, 09/01/12.

Cardiac Remodeling

Cardiac complications of systemic sclerosis. The majority of patients with SSc 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.

Carotid Artery Disease (CAD)

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.

Risk of coronary artery disease (CAD) in patients with systemic sclerosis (SSc): a systematic review and meta-analysis. This study demonstrated a statistically significant increased CAD risk among patients with SSc. PubMed, Clin Rheumatol, 2014 May 25.

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), 2014 Apr;53(4):704-13.

Cholesterol (Better in Systemic Scleroderma Patients)

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)

Conduction Problems

MRI Helps ID Heart Risk in Scleroderma. Among patients with significant arrhythmias seen on Holter monitoring, 73% also had positive gadolinium enhancement on cardiac MRI, compared with only 16% of patients without arrhythmias. MedPageToday, 05/06/2014.

Left Ventricular (LV) Diastolic Dysfunction

Five-year follow-up of left ventricular diastolic function in systemic sclerosis (SSc) patients: determinants of mortality and disease progression. In SSc patients left ventricular diastolic dysfunction is highly prevalent and is associated with increased risk of mortality and the data suggests that in the advanced phase of the disease the myocardial fibrotic processes burns out while the increase of the filling pressure progresses continuously. Seminars in Arthritis and Rheumatism, 11 April 2014.

Echocardiographic follow-up of patients with systemic sclerosis (SSc) by 2D speckle tracking echocardiography (STE) of the left ventricle (LV). STE might be a sensititive and valuable method to detect early LV systolic impairment in patients with SSc and preserved LV ejection fraction during two years and prospective evaluations are needed for prognostic implications of these changes. PubMed, Cardiovasc Ultrasound, 2014 Mar 29;12(1):13.

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

Lipoprotein[a] and Thrombotic Complications

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.

Hypothyroidism Contributes to Increased Triglyceride Levels Among Patients with Systemic Sclerosis (SSc). In a cohort of 49 patients with diffuse SSc clinical and laboratory features of hypothyroidism were observed in 27% of the patients and increased triglyceride levels in more than 55%. Hypothyroidism is a common finding in patients with SSc. J. Rheumatol 2006 April;33:827. Correspondence. Full Text. (Also see Thyroid Disease and Cardiac Involvement)

Mitral and Tricuspid Valve Regurgitation

Mitral Regurgitation. Mitral regurgitation (mitral valve regurgitation, mitral incompetence, mitral insufficiency) is leakage of blood backward through the mitral valve each time the left ventricle contracts. Merck.

Tricuspid Regurgitation. Tricuspid regurgitation (tricuspid incompetence, tricuspid insufficiency) is leakage of blood backward through the tricuspid valve each time the right ventricle contracts. Merck.

Myocardial Ischemia

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).

Recognizing and treating myocarditis in recent-onset systemic sclerosis heart disease. Myocarditis is a common finding in systemic sclerosis (SSc) patients with recent–onset cardiac involvement. PubMed, Semin Arthritis Rheum, 08/06/2013.

Right Ventricular Dysfunction

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.

Six-minute walk test reflects neurohormonal activation and right ventricular function in systemic sclerosis (SSc) patients. Decreased exercise capacity significantly correlates with biochemical and echocardiographic parameters of right ventricular dysfunction and neurohormonal activation providing a potential link for neuroendocrine derangement in patients with SSc. PubMed, Clin Exp Rheumatol, 2013 Feb 13. (Also see Diagnosis of Heart Involvement)

Stroke and Mini-Stroke (Transient Ischemic Attack, or TIA)

Carotid Artery Disease (CAD) is more common in patients with systemic sclerosis and can lead to a stroke. Also, Raynaud's doubles the risk of coronary artery disease and stroke.

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.

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.

Obesity may increase stroke risk in young adults. Obese young adults may be more likely to have a stroke than people who aren't overweight. Reuters Health, 05/15/2015.

Ischaemic Strokes (IS) with Reversible Vasoconstriction (RV) and without Thunderclap Headache: A Variant of the Reversible Cerebral Vasoconstriction Syndrome (RCVS)? RV in our patients differs from the classical characteristics of RCVS by the absence of thunderclap headache, reversible brain oedema and subarachnoid or intracranial haemorrhage. Intracranial arteries should be looked for, by appropriate vascular imaging, in young patients with IS at the acute stage and during the follow-up period. PubMed, Cerebrovasc Dis, 2014 Dec 24;39(1):31-38.

Can You Balance on One Leg? You May Have Lower Stroke Risk. If you can't balance on one leg for at least 20 seconds you may be at risk of a stroke. HealthDay, 12/18/2014.

Neck manipulation may be associated with stroke. Blood vessel tears that lead to strokes may be associated with — but not necessarily caused by — treatments involving neck manipulations, researchers say. Reuters, 08/11/2014.

Both hemorrhagic and ischemic stroke following high doses of cannabis consumption. Symptom onset following unusually high doses of cannabis exposure with the lack of other cardiovascular risk factors supports a causal association of high doses of cannabis consumption in addition to concomitant alcohol and tobacco intake. Analytical observational studies are warranted to establish a cause-and-effect relationship between cannabis use and stroke of hemorrhagic or ischemic origin. EM Consulte, 03/09/2013.

Also see: Antiphospholipid Antibodies, Hypertension, Raynaud's, Scleroderma Brain Involvement and Vasculitis.

Ischemic stroke as the first manifestation of Wegener's granulomatosis. Wegener's granulomatosis should be considered in the differential diagnosis of ischemic stroke even in the absence of extraneurological involvement. PubMed, Rev Med Interne, 2012 Feb;33(2):e6-9. (Also see Wegeners Granulomatosis)

Stroke Patient Stories

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.

Go to Patient and Caregiver Stories of Cardiac Involvement
 
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