GeneHealth - Your precision medicine

Rheumatic Fever

Disease Details

Family Health Simplified

Buy Membership

Description: Rheumatic fever is an inflammatory disease that can develop as a complication of untreated or poorly treated strep throat or scarlet fever caused by Group A Streptococcus bacteria.
Type: Rheumatic fever is an inflammatory disease that can develop as a complication of inadequately treated strep throat or scarlet fever. It is not a genetic condition, so it does not have a type of genetic transmission. Instead, it results from an autoimmune response to infection with Group A Streptococcus bacteria.
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: Rheumatic fever is a serious inflammatory disease that can develop after an infection with Streptococcus bacteria (such as strep throat or scarlet fever).

**Prognosis:**
The prognosis for individuals with rheumatic fever varies based on several factors, including promptness of diagnosis, treatment adherence, and severity of the initial attack. With appropriate medical treatment, including antibiotics to eradicate the streptococcal infection and anti-inflammatory medications, the acute symptoms of rheumatic fever can often be managed effectively. However, long-term outcomes depend significantly on whether there has been damage to the heart, particularly the heart valves, which can lead to rheumatic heart disease. Regular follow-up and preventive measures, such as continuous antibiotic prophylaxis, are crucial to prevent recurrences and minimize complications.

**Nan:**
The term "nan" does not appear to apply to a clinical context for rheumatic fever. If you meant something specific by "nan," please clarify further.
Onset: Rheumatic fever typically develops 1 to 5 weeks after a group A Streptococcal throat infection. Onset is often sudden, with symptoms appearing days to weeks after the initial infection. Common symptoms include fever, joint pain, and inflammation, which can migrate from one joint to another.
Prevalence: The prevalence of rheumatic fever varies widely depending on socioeconomic conditions. In developed countries, it is relatively rare due to better hygiene, living conditions, and access to medical care. However, in developing countries, rheumatic fever remains a significant public health issue, particularly among children and young adults. Accurate prevalence rates are difficult to determine globally, but estimates suggest that millions of people are affected each year, primarily in lower-income regions.
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: Rheumatic fever is not considered intractable. It is a preventable and treatable disease, particularly with early diagnosis and timely management. Treatment typically includes antibiotics to eradicate the streptococcal infection, anti-inflammatory medications to reduce inflammation, and preventive measures, such as continuous antibiotic prophylaxis, to prevent recurrence.
Disease Severity: Rheumatic fever can vary in severity depending on the extent of inflammation and the organs affected, particularly the heart. It can lead to significant complications such as rheumatic heart disease, which can be severe and cause long-term damage to heart valves. Prompt diagnosis and treatment are essential to manage symptoms and prevent complications.
Healthcare Professionals: Disease Ontology ID - DOID:1586
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: Rheumatic fever does not have a carrier status. It is an inflammatory disease that can develop as a complication of inadequately treated strep throat or scarlet fever caused by group A Streptococcus bacteria. It is not a condition one carries; rather, it results from an autoimmune reaction to the bacterial infection.
Mechanism: Rheumatic fever is an inflammatory disease that can develop after an infection with Streptococcus pyogenes (group A streptococcus), which affects the throat. The mechanisms and molecular mechanisms involved include:

### Mechanism:
1. **Infection**: An initial throat infection with group A Streptococcus (GAS) bacterium occurs.
2. **Immune Response**: The immune system responds to the infection by producing antibodies aimed at fighting the bacteria.
3. **Molecular Mimicry**: Some of the streptococcal antigens share structural similarities with human tissue antigens, particularly in the heart, joints, skin, and central nervous system.
4. **Autoimmune Reaction**: The antibodies produced to combat the streptococcal infection mistakenly target human tissues, causing an inflammatory response. This is known as molecular mimicry, where the immune system cannot differentiate between the bacterial antigens and the body's own tissues.

### Molecular Mechanisms:
1. **M protein**: A key virulence factor of GAS is the M protein, which plays a significant role in molecular mimicry. The M protein shares epitopes with myosin (a protein found in the heart), leading to the production of cross-reactive antibodies that attack cardiac tissue.
2. **T Cells Activation**: Cross-reactive T cells are activated and target both the streptococcal antigens and the host's tissues, leading to chronic inflammation.
3. **Cytokine Release**: The activated T cells and autoantibodies lead to the release of pro-inflammatory cytokines and chemokines, which exacerbate tissue damage and inflammation.
4. **Adhesion and Invasion**: GAS has other virulence factors such as exotoxins and enzymes (e.g., streptolysins, hyaluronidase) that contribute to the invasion and adhesion to tissues, perpetuating the immune response.
5. **Complement System**: Activation of the complement system also plays a role, contributing to further tissue injury and inflammation.

These interactions collectively result in the characteristic symptoms of rheumatic fever, which include carditis, arthritis, chorea, subcutaneous nodules, and erythema marginatum. Long-term consequences can lead to rheumatic heart disease, characterized by permanent damage to the heart valves.
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: Compassionate use or expanded access programs allow patients with serious or life-threatening conditions to gain access to investigational treatments outside clinical trials. For rheumatic fever, this approach could involve the use of antibiotics, anti-inflammatory medications, or other treatments that are still under investigation but show promise in managing the disease.

Off-label treatments might include the use of medications approved for other conditions that can help manage rheumatic fever symptoms. For example, corticosteroids or other anti-inflammatory drugs might be used to reduce inflammation and mitigate heart damage.

Experimental treatments for rheumatic fever are typically under investigation in clinical trials. These might include novel anti-inflammatory agents, immune-modulating therapies, or even therapeutic vaccines aimed at preventing group A Streptococcus—the bacteria responsible for triggering the condition.

Physicians prescribing any off-label or experimental treatments must carefully consider the potential benefits and risks, often working within the framework of ethical and legal guidelines.
Lifestyle Recommendations: For rheumatic fever, here are some lifestyle recommendations that can help manage the condition and prevent complications:

1. **Medication Adherence**: Ensure you take all prescribed medications, such as antibiotics, to prevent recurrence and manage symptoms.

2. **Regular Medical Follow-ups**: Attend regular check-ups with your healthcare provider to monitor your condition.

3. **Physical Activity**: Engage in low-impact physical activities as tolerated and advised by your doctor to maintain overall health without overstraining the heart.

4. **Balanced Diet**: Eat a nutritious diet rich in fruits, vegetables, whole grains, and lean proteins to support your immune system and overall health.

5. **Adequate Rest**: Rest is crucial, especially during the acute phase of the disease, to allow your body to heal.

6. **Avoid Smoking and Alcohol**: Smoking and excessive alcohol can exacerbate symptoms and impact overall heart health.

7. **Personal Hygiene**: Maintain good personal hygiene, including regular handwashing, to avoid infections that could trigger rheumatic fever.

8. **Stay Informed**: Educate yourself about rheumatic fever to better understand your condition and the importance of preventative steps.

9. **Vaccinations**: Stay up-to-date with recommended vaccinations, as infections can worsen the condition.

Consult your healthcare provider for personalized advice tailored to your specific situation.
Medication: For rheumatic fever, the primary medications commonly used include:

1. **Antibiotics:** Typically penicillin or other suitable antibiotics to eradicate the streptococcal bacteria.
2. **Anti-inflammatory agents:** Aspirin, or in some cases, corticosteroids, to reduce inflammation and relieve symptoms such as arthritis or carditis.

Ongoing prophylactic antibiotics may be recommended to prevent recurrence, depending on individual risk factors.
Repurposable Drugs: For rheumatic fever, repurposable drugs would include:

1. **Aspirin** - An anti-inflammatory that can help reduce inflammation and manage pain associated with rheumatic fever.
2. **Penicillin** - An antibiotic commonly used to treat the underlying streptococcal infection causing the disease.
3. **Corticosteroids (e.g., Prednisone)** - These can be used to manage severe inflammation and carditis.
4. **NSAIDs (e.g., Ibuprofen)** - Non-steroidal anti-inflammatory drugs can help manage pain and inflammation.
5. **Diuretics (e.g., Furosemide)** - These may be used in cases of severe carditis to help manage symptoms of heart failure.
Metabolites: Rheumatic fever is an inflammatory disease that can develop as a complication of inadequately treated strep throat or scarlet fever caused by Streptococcus pyogenes bacteria. There are no specific metabolites directly associated with rheumatic fever. Diagnosis is typically based on clinical criteria and testing for evidence of prior streptococcal infection.
Nutraceuticals: There is no strong scientific evidence to support the efficacy of nutraceuticals in the treatment of rheumatic fever. The condition is typically managed through antibiotics to treat the underlying streptococcal infection, along with anti-inflammatory medications such as aspirin or corticosteroids to reduce inflammation and manage symptoms. It's essential to follow a healthcare provider's recommendations for treatment.
Peptides: Rheumatic fever is an inflammatory disease that can develop as a complication of untreated or poorly treated streptococcal throat infection. It primarily affects the heart, joints, skin, and brain. The relationship with "peptides, nan" is not clear or specific to the disease. Peptides are short chains of amino acids, and while they play various roles in biological processes, there's no direct or well-known connection between peptides and the nanoscale specifically in the context of rheumatic fever. Further context or clarification may be needed for a more precise explanation.