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Heart Involvement in Systemic Rheumatic Diseases

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This article explains the involvement of the heart in systemic rheumatic disease and explores its effect and clinical significance.

Written by

Dr. Leenus A. E

Medically reviewed by

Dr. Yash Kathuria

Published At August 22, 2023
Reviewed AtDecember 1, 2023

Introduction

Rheumatic diseases are characterized by joint and muscular discomfort, edema, and inflammation. It is typical of rheumatic disorders for the immune system to be involved. More than 200 diseases are included in these systemic rheumatic diseases, the most prevalent of which are osteoarthritis, arthritis, lupus, and ankylosing spondylitis. However, the most frequent rheumatic disease diagnosed in patients in intensive care units is rheumatoid arthritis. Systemic lupus erythematosus and systemic sclerosis are then diagnosed less frequently. Rheumatic disorders affect the joints, muscles, and connective tissues, thereby affecting the entire musculoskeletal system. Complex clinical and pharmaceutical histories and multi-organ system pathology are seen in patients with systemic rheumatic diseases.

How Is Heart Involved in Systemic Rheumatic Diseases?

The cardiac vasculature, valves, myocardium (the muscular tissues of the heart), pericardium (protective, fluid-filled sac around the heart), and conduction system can all be impacted by autoimmune rheumatic disorders, which can result in a wide range of cardiovascular symptoms that might be clinically asymptomatic or cause significant cardiovascular morbidity and mortality.

What Are the Heart Conditions in Systemic Rheumatic Disease?

1) Atherosclerosis

Although the mechanisms underlying accelerated atherosclerosis are poorly understood, chronic inflammation has been proposed as a risk factor for the onset of atherosclerotic disease. While there are differences between specific rheumatological conditions, chronic inflammation is common. Tumor necrosis factor (TNF), a cytokine (protein controlling inflammation), is also linked to elevated dyslipidemia (imbalance of lipids levels), insulin resistance, a prothrombotic state, and the activation of the inflammatory cascade, which leads to an increase in prostaglandins, matrix metalloproteinases, IL-1, IL-6, and other inflammatory mediators that may hasten atherosclerosis. The high risk of atherosclerosis is assumed to be caused by chronic tissue-destructive inflammation, which is regarded to be a common symptom of rheumatic disease patients with dysfunctioning immune responses.

(i) Rheumatoid Arthritis (RA)

Cardiovascular disease, particularly coronary artery disease, is more likely to occur in RA patients. Additionally, it was found that patients with RA had a greater case fatality rate following acute myocardial infarctions than patients without RA. Due to their chronic inflammation, patients with RA also have dyslipidemia, which includes low HDL cholesterol and high levels of tiny LDL cholesterol.

(ii) Systemic Sclerosis (SSc)

Patients with systemic sclerosis (SSc) may be at higher risk for atherosclerosis because of alterations in the arterial wall in addition to RA and systemic lupus erythematosus. Endothelial damage and decreased oxygen delivery to tissues are the underlying reasons for atherosclerosis in SSc patients.

(iii) Vasculitis

Immune-mediated rheumatological conditions known as systemic vasculitides, including giant cell arteritis, Takayasu arteritis, polyarteritis nodosa, and anti-neutrophil cytoplasmic antibody-associated vasculitis, cause inflammation of the vasculature and hasten atherosclerosis.

2) Pericarditis

(i) Rheumatoid Arthritis

Pericarditis is a frequent symptom of rheumatic diseases typically caused by underlying inflammation. Pericarditis is a frequent symptom of rheumatic diseases typically caused by underlying inflammation. Pericarditis is the most frequent heart symptom in RA patients. Especially pericarditis is the most frequent heart symptom in RA patients. Immune complexes and rheumatoid factors can be identified in the pericardial fluid, which is characterized by neutrophil infiltrates, high protein levels, low glucose levels, and low complement levels. Chronic pericarditis has the potential to lead to pericardial calcification. Patients with RA may also develop constrictive pericarditis; however, this condition is typically clinically quiet.

(ii) Systemic Lupus Erythematosus (SLE)

The most common clinical cardiovascular symptom of SLE is pericardial dysfunction. The most typical cardiac symptoms of SLE include pericarditis and pericardial effusion. Chest pain is the most frequent patient symptom.

3) Coronary Artery Disease

Patients with RA are more prone to ventricular arrhythmias and sudden cardiac mortality due to underlying coronary artery disease. It is also believed that rheumatoid nodules increase patients' risk of heart involvement.

4) Valvular Manifestations

Patients with rheumatological diseases, notably those with RA, SLE, or ankylosing spondylitis, may experience valvular symptoms. Valvular calcifications can impact up to 80 percent of individuals, and studies have indicated that patients with RA and SLE are more likely to have them. Similar processes may mediate this effect to those causing accelerated atherosclerosis.

(i) Rheumatoid Arthritis

Comparing RA patients to the general population, valvular disease is more common in RA patients. In around 30 percent of RA patients, the valvular disease can be detected by echocardiography, but it is typically clinically silent.

(ii) SLE

Endocarditis and the development of valve nodules are both linked to SLE. Despite being very common in autopsy reports of SLE patients, valvular nodules are not clinically significant. In studies, nonbacterial vegetations, also known as Libman-Sacks vegetations, have been found in up to 60% of SLE patients. These vegetations, found in up to 15 percent of mitral and 19 percent of aortic valves, are typically univalvular, tiny, and left-handed.

5) Myocardial Manifestations

In addition to directly affecting the myocardium, autoimmune rheumatic disorders can cause myocarditis or myocardial dysfunction due to several inflammatory and autoimmune pathways. Independent of the usual risk factors for cardiovascular diseases, patients with RA have an elevated risk of myocardial dysfunction and congestive heart failure. Furthermore, congestive heart failure was more likely to occur in rheumatoid factor-positive patients. Despite being a frequent symptom of some rheumatic diseases, myocarditis is hardly seen in SLE patients. Myocardial fibrosis, however, is a significant SSc cardiac symptom.

How Is Heart Involvement in Systemic Rheumatic Disease Treated?

It has been hypothesized that several medications are suitable for treating cardiovascular disease in people with autoimmune illnesses. In numerous trials, disease-modifying antirheumatic medications (DMARDs) were found to slow the development of atherosclerosis in RA patients. A class of pharmaceuticals known as disease-modifying antirheumatic drugs (DMARDs) is frequently prescribed to persons with rheumatoid arthritis. Some medications are also used to treat other diseases, such as systemic lupus erythematosus, psoriatic arthritis, and ankylosing spondylitis. DMARDs suppress the hyperactive immunological and inflammatory systems. They are not intended to offer symptom relief immediately but take effect over weeks or months.

Conclusion

Patients with systemic autoimmune rheumatic diseases must have the proper management due to their elevated risk of cardiovascular disease. Despite the absence of established risk factors, adherence to primary prevention recommendations is essential, given the elevated risk of early atherosclerosis in RA patients. Since autoimmune rheumatic disorders have been linked to cardiovascular morbidity and mortality, managing and treating individuals with these diseases is essential. With the emergence of a number of specialized clinics around the world, the area of cardio rheumatology is growing as evidence of the rising need for cardiovascular care among patients with autoimmune inflammatory rheumatological diseases.

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Dr. Yash Kathuria
Dr. Yash Kathuria

Family Physician

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