Cardiovascular Complications of Adult-Onset Still's Disease

Introduction:

A great challenge that the healthcare industry faces today is the diagnosis and treatment of rare inflammatory diseases before the occurrence of any severe complications. Inflammatory diseases are a group of conditions that occur when the body's inflammatory mechanisms go overboard to cause disease in an individual. One such rare inflammatory disease is the adult-onset Still's disease, which affects the body’s joints.

What Is Adult-Onset Stills Disease?

Adult-onset Still's disease is a generalized autoinflammatory condition that affects the joints in the body. There is persistent and chronic inflammation in the body. This can cause high spiking fever, rashes (salmon-colored), and arthritis (joint inflammation with pain). The body's main target for this autoinflammation process is the musculoskeletal system. However, when all the organs are affected, serious complications occur.

What Are the Cardiovascular Complications of Adult-Onset Stills Disease?

In adult-onset Still's disease, there is a high release of inflammatory mediators like cytokines. These are responsible for the causation of cardiovascular complications.

Factors Causing Cardiovascular Inflammation:

• Proinflammatory cytokines like interleukins 1 and 6 (IL-1 and IL-6) are high in adult-onset Still's disease. This causes fever and other systemic manifestations of Still's disease.

• Such inflammation over a chronic period can cause impairment of the endothelium (innermost layer of the blood vessel). This can predispose the individual to develop thrombosis and other cardiovascular abnormalities.

Common Cardiovascular Complications:

• Pericardial inflammation (inflammation of the sac surrounding the heart) is the main feature of adult-onset Still's disease. When the pericardium is inflamed, it will cause breathing issues and chest pain. When the condition persists for a chronic period, it can lead to fluid accumulation and a serious condition called cardiac tamponade (the heart will be compressed due to external pressure by the accumulated fluids).

• The musculoskeletal system throughout the body is affected in adult-onset Still’s disease. This involves the cardiac muscles as well. Inflammation of the cardiac muscles (myocarditis) can impair blood supply to the heart and other organs. A dysfunctional blood supply will, in turn, cause breathing problems and generalized fatigue and, in severe cases, can fail vital body organs.

• Vasculitis occurs as a result of the continued secretion of inflammatory mediators. This predisposes the formation of clots, thus resulting in thrombosis and embolism-related complications.

• When the levels of inflammatory mediators go high, the lipid metabolism in the body is affected. This causes cholesterol plaques to accumulate in the vessel walls and lining (atherosclerosis). As a result, blood supply flow to vital organs, including the heart, is compromised. This can lead to ischemic attacks and stroke.

• In some instances, this chronic autoinflammatory disease can also affect the coronary arteries (the vessels that supply blood to the heart muscle). Coronary artery involvement can lead to severe complications like vasculitis or aneurysm formation, increasing the risk of myocardial infarction (heart attack).

How to Diagnose Cardiovascular Complications Due to Stills Disease?

Still’s disease is primarily identified and diagnosed by the presence of persistent high fever with rashes all over the body and arthritic pain. Cardiovascular complications should be diagnosed at an early stage. Common diagnostic modalities employed are:

• Echocardiography.

• Electrocardiogram (ECG).

• Complete blood count. This shows increased circulating white blood cells and reduced erythrocytes (red blood cells).

• C-reactive protein and erythrocyte sedimentation rate (ESR) will be high.

• Magnetic resonance imaging is a significant tool to identify the extent of damage incurred to blood vessels and cardiac muscles.

• In recent days, cardiac biomarkers have been widely used for precise and timely diagnosis of cardiovascular disorders and dysfunctions.

How Are Cardiovascular Complications Treated?

Managing cardiovascular complications in adult-onset Still’s disease involves a comprehensive, multidisciplinary approach.

• Controlling systemic inflammation is a key aspect of treatment. Nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, and disease-modifying antirheumatic drugs (DMARDs) like Methotrexate are commonly used to manage the inflammatory aspects of adult-onset Still’s disease.

• Understanding the mechanism of causation of cardiovascular disorders in adult-onset Still's disease is important to treat the complications and the symptoms adeptly.

• Since adult-onset Still's disease is a condition caused by inflammatory mediators, modulating them is the right way of treatment. Hence, immunotherapy or biological therapy has emerged as the latest treatment option. In this case, the objective is to target the mediators, especially interleukin 1 and 6. This has shown better results in subsiding the symptoms and improving the prognosis of the condition. This also proved to reduce the incidence of cardiovascular complications.

• Medications such as Anakinra and Tocilizumab may be considered in refractory cases. These medications block the receptors for the action of interleukin-1 and thus prevent the inflammatory cascade responsible for the incidence of adult-onset Still's disease.

What Is the Prognosis of Adult-Onset Stills Disease After Cardiovascular Complications?

The prognosis can vary extensively depending on the severity, involvement of the cardiovascular system, and the duration taken to treat the complications. Identifying and treating cardiovascular complications of adult-onset Still's syndrome requires comprehensive evaluation and treatment plans by cardiologists and rheumatologists.

Conditions like pericarditis and myocarditis, if effectively managed, may result in better outcomes. However, more severe complications, such as widespread vasculitis or coronary artery involvement, may pose greater challenges.

Also, if the inflammation part of the disease is controlled effectively, there is a great way to minimize cardiovascular complications. This should be done through timely administration of medicines and continuous monitoring for cardiovascular involvement.

Individuals with adult-onset Still's disease are also susceptible to developing traditional risk factors for cardiovascular diseases like fluctuating blood pressure, heart rate, and hypercholesterolemia. This should be diagnosed early and treated effectively for a better prognosis.

Above all, long-lasting monitoring is essential. This will ensure no complications arise, and if any complications occur, timely treatment can be done.

Conclusion:

Overall, while adult-onset Still's disease primarily presents as a systemic inflammatory disorder affecting the joints, its impact extends to the cardiovascular system, leading to complications such as pericarditis, myocarditis, vasculitis, and coronary artery involvement. The inflammatory nature of this disease, marked by the release of proinflammatory cytokines, contributes to endothelial dysfunction and cardiovascular manifestations. If all these are controlled with the help of a team of rheumatologists and cardiologists, the prognosis is better.