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Chronic Rheumatic Pericarditis

Disease Details

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Description: Chronic rheumatic pericarditis is long-term inflammation of the pericardium due to rheumatic fever, often leading to thickening and scarring of the pericardial sac.
Type: Chronic rheumatic pericarditis is not classified as a genetic disorder. It is a type of pericarditis, which is inflammation of the pericardium, and it results from the long-term damage caused by rheumatic fever, a complication of Streptococcus pyogenes (group A streptococcus) infection. Therefore, there is no genetic transmission associated with this condition.
Signs And Symptoms: The disease typically develops two to four weeks after a throat infection. Symptoms include: fever, painful joints with those joints affected changing with time, involuntary muscle movements, and occasionally a characteristic non-itchy rash known as erythema marginatum. The heart is involved in about half of the cases. Damage to the heart valves usually occurs only after several attacks but may occasionally occur after a single case of RF. The damaged valves may result in heart failure and also increase the risk of atrial fibrillation and infection of the valves.
Prognosis: Prognosis for chronic rheumatic pericarditis can vary based on the severity of the condition and the response to treatment. Generally, the prognosis is guarded as it may lead to complications such as constrictive pericarditis, which can significantly impact cardiac function. Early diagnosis and appropriate management are crucial to improving outcomes.
Onset: Chronic rheumatic pericarditis is a condition characterized by long-term inflammation of the pericardium resulting from rheumatic fever. Rheumatic fever, an inflammatory disease that may follow a Streptococcus pyogenes infection, primarily affects young people between the ages of 5 and 15. The onset of chronic rheumatic pericarditis can vary, often emerging years after the initial rheumatic fever episode. The condition develops gradually as the inflammation persists and progresses, leading to fibrosis and thickening of the pericardium.
Prevalence: There is limited specific data on the precise prevalence of chronic rheumatic pericarditis. However, rheumatic heart disease, which can lead to pericarditis, is more prevalent in developing countries and affects approximately 32 million people worldwide. The prevalence of chronic rheumatic pericarditis specifically would be a subset of these cases, but exact figures are not commonly reported.
Epidemiology: About 33 million people are affected by rheumatic heart disease with an additional 47 million having asymptomatic damage to their heart valves. As of 2010 globally it resulted in 345,000 deaths, down from 463,000 in 1990.In Western countries, rheumatic fever has become fairly rare since the 1960s, probably due to the widespread use of antibiotics to treat streptococcus infections. While it has been far less common in the United States since the beginning of the 20th century, there have been a few outbreaks since the 1980s. The disease is most common among Indigenous Australians (particularly in central and northern Australia), Māori, and Pacific Islanders, and is also common in Sub-Saharan Africa, Latin America, the Indian subcontinent, and North Africa.Rheumatic fever primarily affects children between ages 5 and 17 years and occurs approximately 20 days after strep throat. In up to a third of cases, the underlying strep infection may not have caused any symptoms.The rate of development of rheumatic fever in individuals with untreated strep infection is estimated to be 3%. The incidence of recurrence with a subsequent untreated infection is substantially greater (about 50%). The rate of development is far lower in individuals who have received antibiotic treatment. Persons who have had a case of rheumatic fever have a tendency to develop flare-ups with repeated strep infections.The recurrence of rheumatic fever is relatively common in the absence of maintenance of low dose antibiotics, especially during the first three to five years after the first episode. Recurrent bouts of rheumatic fever can lead to valvular heart disease. Heart complications may be long-term and severe, particularly if valves are involved. In countries in Southeast-Asia, sub-Saharan Africa, and Oceania, the percentage of people with rheumatic heart disease detected by listening to the heart was 2.9 per 1000 children and by echocardiography it was 12.9 per 1000 children. To assist in the identification of RHD in low resource settings and where prevalence of GAS infections is high, the World Heart Federation has developed criteria for RHD diagnosis using echocardiography, supported by clinical history if available. The WHF additionally defines criteria for use in people younger than age 20 to diagnose "borderline" RHD, as identification of cases of RHD among children is a priority to prevent complications and progression. However, spontaneous regression is more likely in borderline RHD than in definite cases, and its natural history may vary between populations.Echocardiographic screening among children and timely initiation of secondary antibiotic prophylaxis in children with evidence of early stages of rheumatic heart disease may be effective to reduce the burden of rheumatic heart disease in endemic regions. The efficacy of treating latent RHD in populations with high prevalence is balanced by the potential development of antibiotic resistance, which might be offset through use of narrow-spectrum antibiotics like benzathine benzapenicillin. Public health research is ongoing to determine if screening is beneficial and cost effective.
Intractability: Chronic rheumatic pericarditis is not typically considered intractable. With appropriate medical management, including anti-inflammatory medications and treatment of the underlying rheumatic condition, symptoms can often be controlled. However, complications such as constrictive pericarditis may require more intensive interventions, including surgery.
Disease Severity: Chronic rheumatic pericarditis is a long-term inflammation of the pericardium (the sac surrounding the heart) that results from rheumatic fever. The severity of this condition can vary. Some individuals may experience mild symptoms and stable disease, while others may suffer from significant complications, including pericardial thickening, constrictive pericarditis, and heart dysfunction. Regular medical follow-up is essential to manage and monitor the condition.
Healthcare Professionals: Disease Ontology ID - DOID:1869
Pathophysiology: Rheumatic fever is a systemic disease affecting the connective tissue around arterioles, and can occur after an untreated strep throat infection, specifically due to group A streptococcus (GAS), Streptococcus pyogenes. The similarity between antigens of Streptococcus pyogenes and multiple cardiac proteins can cause a life-threatening type II hypersensitivity reaction. Usually, self reactive B cells remain anergic in the periphery without T cell co-stimulation. During a streptococcal infection, mature antigen-presenting cells such as B cells present the bacterial antigen to CD4+T cells which differentiate into helper T2 cells. Helper T2 cells subsequently activate the B cells to become plasma cells and induce the production of antibodies against the cell wall of Streptococcus. However the antibodies may also react against the myocardium and joints, producing the symptoms of rheumatic fever. S. pyogenes is a species of aerobic, cocci, gram-positive bacteria that are non-motile, non-spore forming, and forms chains and large colonies.S. pyogenes has a cell wall composed of branched polymers which sometimes contain M protein, a virulence factor that is highly antigenic. The antibodies which the immune system generates against the M protein may cross-react with heart muscle cell protein myosin, heart muscle glycogen and smooth muscle cells of arteries, inducing cytokine release and tissue destruction. However, the only proven cross-reaction is with perivascular connective tissue. This inflammation occurs through direct attachment of complement and Fc receptor-mediated recruitment of neutrophils and macrophages. Characteristic Aschoff bodies, composed of swollen eosinophilic collagen surrounded by lymphocytes and macrophages can be seen on light microscopy. The larger macrophages may become Anitschkow cells or Aschoff giant cells. Rheumatic valvular lesions may also involve a cell-mediated immunity reaction as these lesions predominantly contain T-helper cells and macrophages.In rheumatic fever, these lesions can be found in any layer of the heart causing different types of carditis. The inflammation may cause a serofibrinous pericardial exudate described as "bread-and-butter" pericarditis, which usually resolves without sequelae. Involvement of the endocardium typically results in fibrinoid necrosis and wart formation along the lines of closure of the left-sided heart valves. Warty projections arise from the deposition, while subendocardial lesions may induce irregular thickenings called MacCallum plaques.
Carrier Status: Chronic rheumatic pericarditis is a condition characterized by long-term inflammation of the pericardium, the membrane surrounding the heart, due to rheumatic fever. It is not associated with a carrier status as it is not a genetic condition nor a transmissible disease. Instead, it results from an autoimmune reaction to a previous streptococcal infection.
Mechanism: Chronic rheumatic pericarditis is a long-term inflammation of the pericardium, the protective sac surrounding the heart, usually resulting from rheumatic fever.

**Mechanism:**
1. **Inflammation**: Rheumatic fever, typically following a Streptococcus pyogenes (Group A Streptococcus) infection, leads to an autoimmune response where the body's immune system attacks its own tissues, including the heart and pericardium.
2. **Fibrosis**: Chronic inflammation causes scarring and thickening of the pericardium (fibrosis). This can lead to constriction of the heart, known as constrictive pericarditis, impairing its function.
3. **Calcification**: Prolonged inflammation might also lead to calcification of the pericardium, further restricting heart movements.

**Molecular Mechanisms:**
1. **Molecular Mimicry**: Antibodies generated against streptococcal M protein cross-react with similar-looking proteins in the heart tissue, triggering an autoimmune response.
2. **Cytokine Release**: Inflammation involves the release of inflammatory cytokines like TNF-α, IL-1, and IL-6, which recruit immune cells to the pericardium and perpetuate tissue damage.
3. **Matrix Remodeling**: Chronic inflammation results in the activation of fibroblasts and other cells that produce extracellular matrix proteins, causing fibrotic remodeling of the pericardial tissue.
4. **Reactive Oxygen Species (ROS)**: Elevated ROS levels during inflammation lead to oxidative stress, further damaging pericardial tissues and contributing to fibrosis.
5. **Autoimmune Reactivity**: T-cells and other immune components may misrecognize pericardial cells as foreign, continuing the cycle of inflammation and damage.

Managing chronic rheumatic pericarditis often requires addressing the underlying inflammation and autoimmune dysregulation to prevent long-term complications.
Treatment: The management of rheumatic fever is directed toward the reduction of inflammation with anti-inflammatory medications such as aspirin or corticosteroids. Individuals with positive cultures for strep throat should also be treated with antibiotics.
Compassionate Use Treatment: Chronic rheumatic pericarditis primarily involves inflammation of the pericardium due to rheumatic fever. Compassionate use or experimental treatments for this condition may not be widely documented, as established therapies generally focus on managing inflammation and symptoms. However, off-label use of medications such as colchicine, typically used for gout, has been explored to reduce inflammation and prevent recurrence in pericarditis. Investigational therapies might include novel anti-inflammatory agents or biologics targeting specific pathways involved in chronic inflammation. Clinical trials could be a potential route for accessing experimental treatments, under the guidance of a healthcare provider.
Lifestyle Recommendations: Lifestyle recommendations for chronic rheumatic pericarditis include:

1. **Regular Medical Follow-up**: Ensure consistent follow-ups with a cardiologist to monitor the condition and adjust treatments as necessary.
2. **Medication Adherence**: Take prescribed medications as directed to manage symptoms and prevent complications.
3. **Balanced Diet**: Maintain a heart-healthy diet rich in fruits, vegetables, lean proteins, and whole grains while limiting salt intake.
4. **Regular Physical Activity**: Engage in moderate exercise as recommended by your healthcare provider, but avoid strenuous activities that may exacerbate symptoms.
5. **Stress Management**: Practice stress-reducing techniques such as yoga, meditation, or deep-breathing exercises.
6. **Avoid Smoking and Alcohol**: Refrain from smoking and limit alcohol consumption as they can aggravate heart conditions.
7. **Stay Hydrated**: Drink plenty of water unless advised otherwise by your doctor to manage fluid retention.
8. **Weight Management**: Maintain a healthy weight to reduce the strain on your heart.
9. **Recognize Symptoms**: Be aware of symptoms like chest pain, shortness of breath, or fatigue, and seek medical attention if they worsen.
10. **Vaccinations**: Stay up-to-date with vaccinations, particularly for influenza and pneumococcal infections, to avoid respiratory complications.
Medication: Chronic rheumatic pericarditis typically involves inflammation and scarring of the pericardium due to rheumatic fever.

Medication: Treatment often includes anti-inflammatory drugs, such as nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen or aspirin. Corticosteroids may be prescribed in more severe cases. Additionally, patients may need antibiotics to address any underlying streptococcal infection that could have triggered rheumatic fever.

If you need specific information on medication or treatment options, consulting with a healthcare provider is essential for tailored medical advice.
Repurposable Drugs: There is limited information regarding repurposable drugs specifically for chronic rheumatic pericarditis. Treatment generally focuses on managing symptoms and preventing complications associated with the condition. Common medications include anti-inflammatory agents, such as nonsteroidal anti-inflammatory drugs (NSAIDs) or colchicine, which are used to reduce inflammation and pain.

In some cases, corticosteroids might be used if the patient does not respond to NSAIDs or colchicine. Additionally, immunosuppressive drugs may be considered in certain situations. Antibiotics might be prescribed if an underlying bacterial infection is identified.

Further clinical trials and research are needed to identify specific repurposable drugs for chronic rheumatic pericarditis. If you are seeking treatment options, it's important to consult a healthcare provider specializing in this condition.
Metabolites: Chronic rheumatic pericarditis is a condition characterized by long-term inflammation of the pericardium due to rheumatic fever. While specific metabolites directly associated with chronic rheumatic pericarditis are not well-defined, general metabolic changes may occur due to the underlying inflammatory and immune processes. These may include altered levels of:

1. C-reactive protein (CRP) – an inflammatory marker.
2. Erythrocyte sedimentation rate (ESR) – another marker of inflammation.
3. Cytokines – such as interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-α), which are involved in the inflammatory response.
4. Autoantibodies – produced as part of the autoimmune response in rheumatic fever.

Further research specific to metabolomic profiling in chronic rheumatic pericarditis may provide more detailed insights.
Nutraceuticals: Nutraceuticals have not been shown to have a specific role in the treatment or management of chronic rheumatic pericarditis. It's critical for patients to follow medical advice and treatments prescribed by healthcare professionals for this condition.
Peptides: For chronic rheumatic pericarditis, peptides and nanoparticles (nan) can potentially be involved in innovative treatments or diagnostic methods. Researchers are exploring the use of specific peptides that modulate the immune response or reduce inflammation. Nanoparticles may serve as drug delivery systems, allowing for targeted therapy with reduced side effects. However, these approaches are still under investigation and not yet widely adopted in standard clinical practice.