Systemic Scleroderma Cardiac (Heart) Involvement

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Aortic Stiffness
Asymptomatic Pericardial
Cardiac Remodeling
Carotid Artery Disease
Left Ventricle
Lipoprotein[a], Thrombotic
Mitral/Tricuspid Valve
Myocardial Ischemia
Right Ventricle
Stroke and TIA


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)

Incidental significant arrhythmia in scleroderma (SSC) associates with cardiac magnetic resonance measure of fibrosis and hs-TnI and NT-proBNP. This first implantable loop recorder study identified potentially life-threatening arrhythmias in asymptomatic SSc patients attributable to a primary SSc heart disease. PubMed, Rheumatology (Oxford), 2019 Jul 1;58(7):1221-1226. (Also see Scleroderma Biomarkers)

Cardiac phenotype in mouse models of systemic autoimmunity. For this Review, we systematically collated information on the cardiac phenotype in the most common inducible, spontaneous and engineered mouse models of systemic lupus erythematosus, rheumatoid arthritis and systemic sclerosis. PubMed, Dis Model Mech, 2019 Mar 8;12(3).

Bundle Branch Blocks (BBs) and Fragmented QRS Complex in Iranian Patients with Systemic Sclerosis (SSc). BBBs and the fQRS were more prevalent in our patients with SSc, without any association with the involvement of the other organs. PubMed, J Tehran Heart Cent, 2019 Jan;14(1):6-11.

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.

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.

APS and Heart Disease
Scleroderma and APS
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.


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)

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.

Cardiac Remodeling

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).

Carotid Artery Disease (CAD)

Myocardial fibrosis detected by magnetic resonance imaging, elevated U-CRP and higher mRSS are predictors of cardiovascular complications in systemic sclerosis (SSc) patients. This study shows that myocardial fibrosis, elevated ultrasensitive CRP, and higher maximum mRSS are independent predictors of cardiovascular outcomes in SSc patients. PubMed, Semin Arthritis Rheum, 02/11/2019.

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).

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)

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.

Left Ventricular (LV) Diastolic Dysfunction

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

Lipoprotein[a] and Thrombotic Complications

Case Reports: Thrombotic complications after radial arterial line placement in systemic sclerosis. 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. PubMed, Semin Arthritis Rheum.

Mitral and Tricuspid Valve Regurgitation

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.

Case Report: A case of overlap syndrome (scleroderma and polymyositis) associated with the development of sudden chest pain due to myocardial damage. Synthetic judgement of serum brain natriuretic peptide/ troponin T levels and cardiac MRI is useful in the search for the cause of chest symptoms even in patients with collagen diseases. PubMed, Drug Discov Ther, 2019;13(5):297-298. (Also see Symptoms/Complications of Polymyositis and Scleroderma in Overlap)

Clinical and laboratory predictions of myocardial inflammation as detected by cardiac magnetic resonance imaging in patients with systemic sclerosis: A pilot study. An increased risk of myocardial inflammation was associated with young age and high modified Rodnan skin score at onset. PubMed, Int J Rheum Dis, 10/29/2019.

Right Ventricular Dysfunction

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)

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.

How Artificial Intelligence (AI) Can Predict Heart Attacks and Strokes. AI is making its way into health care, and one of its first stops is making sense of all of those scans that doctors” order. Times Healthland, 02/14/2020.

Increased Risk of Ischemic Stroke in Systemic Sclerosis (SSc): A National Cohort Study of US Veterans. Patients with SSc represent a population likely to benefit from targeted stroke screening or prevention therapies. PubMed, J Rheumatol, 2020 Jan;47(1):82-88.

Stroke recovery clue from patient walking speed. World-leading research is helping scientists find new ways of trying to help younger people who have had a stroke get back to work. BBC Health News, 10/28/2019.

Smokers have higher risk for multiple strokes. Smokers who have a stroke are much more likely to have another one if they don’t quit or at least cut back, a Chinese study suggests. Reuters Health, 05/17/2019.

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.

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)

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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