Kembara Xtra - Medicine - Rheumatic Fever

​Kembara Xtra - Medicine -  Rheumatic Fever 
The autoimmune and inflammatory reaction known as acute rheumatic fever (ARF) is caused by an infection caused by group A streptococcus (GAS) and can have an effect on more than one organ system.
Rheumatic heart disease (RHD) is a form of chronic heart disease that can result from acute disease that is not treated.
In the absence of appropriate antibiotic treatment, recurrence is rather common.


Considerations Relating to Children

The majority of instances are found in children between the ages of 5 and 15, while cases in children younger than 5 are extremely rare.


In terms of epidemiology, ARF and RHD are primarily confined to countries with low incomes as well as marginalized sectors of countries with higher incomes.
 Male = female; the risk of developing chorea and RHD is higher in females. Endemic regions include the South Pacific, indigenous populations of Australia and New Zealand, Africa, and Asia. The overall incidence of the disease has been on the decline for the past 25 years. The vast majority of newly reported cases are found in developing nations. The mean incidence worldwide varies from 8 to 51 cases per 100,000 school-aged children, but in endemic locations, prevalence can be more than 1,000 cases per 100,000 people.
 The incidence of ARF in school-aged children in the United States is roughly 0.6–3.4 cases per 100,000 students.

In emerging regions, RHD affects more than 33 million people and is the major cause of death from cardiovascular disease in the first half of a person's life. The prevalence of RHD has been increasing as a result of advances in medical treatment and increased life expectancy.

Causes and effects: etiology and pathophysiology 

 ARF often manifests as two to three weeks following an infection with GAS pharyngitis; however, GAS impetigo may also serve as a prior infection in some cases.
Despite the fact that pathogenicity is not fully understood, there is widespread agreement among experts that genetic and molecular mimicry that results in an inflammatory cascade is a critical step in the progression of the disease. Moreover, joint involvement is most likely due to the accumulation of immune complexes.
ARF is inherited, has several genes responsible for its development, and shows variable and incomplete penetrance. Certain native populations have a higher risk of developing the disease.


Risk Factors 
The most significant risk factors include financial hardship, overcrowding in the home, genetic susceptibility, racial or ethnic predisposition, and social inferiority.


General Prevention 
 
Primary prevention: ARF can be avoided in the majority of patients by receiving the proper therapy for a streptococcal infection.
 Long-term antibiotic prophylaxis (up to 5 to 10 years), also known as secondary prevention, for the purpose of preventing recurrence. Diagnosis
 Jones, Most Recent Edition, 2015 For the purpose of diagnosis, criteria and laboratory evidence of a previous GAS infection are utilized.
 Initial ARF: either two majors or one major and two minors
 ARF that recurs: either two major or one major and two minor or three minor
 The presence of Sydenham chorea is sufficient on its own to diagnose ARF, even in the absence of laboratory evidence of an infection that came before.

Low risk population 
Major criteria : 
Carditis ( clinical or subclinical) 
Polyarthritis 
Chorea 
Erythema Marginatum
Subcutaneous Nodules 

Minor Criteria:
Polyarthralgia 
Fever equal or more than 38.5 Celsius 
ESR equal or more than 60mm/hr
CRP equal or more than 3mg/dl
Increased PR interval 

Moderate and High Risk Population 
Major criteria : 
Carditis ( clinical or subclinical) 
Polyarthritis or monoarthritic 
Chorea 
Erythema Marginatum
Subcutaneous Nodules 

Minor Criteria:
Monoarthralgia 
Fever equal or more than 38.0Celsius 
ESR equal or more than 30mm/hr
CRP equal or more than 3mg/dl
Increased PR interval


Histopathology 
The symptoms of ARF usually appear two to three weeks after a bout of GAS pharyngitis or impetigo.
 Polyarthritis, which affects the knees, ankles, elbows, and wrists, accounts for 35–66% of cases and is frequently the first symptom; individual cases improve within a few days and appear to "migrate." Aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs) lead to rapid relief but may also disguise initial symptoms.
Patients at moderate or high risk may simply have a single joint affected by osteoarthritis.
 High fever
,rash known as erythema marginatum,
 pancarditis or valvulitis can be subclinical (asymptomatic and without auscultatory signs) or clinically obvious in between 50 and 70 percent of patients.
 


Sydenham chorea (late manifestation [1 to 6 months after infection in 10–30%): motions that are purposeless, uncontrollable, and nonstereotypical, most usually involving the face and hands.
- More prevalent in females between the ages of 5 and 15
– May become better during sleep or go away altogether – May be connected with muscular weakness, which may show up as a decline in handwriting quality
– It is possible that psychiatric symptoms, such as emotional lability and obsessive-compulsive symptoms, would first appear before chorea.
— May be self-resolved over a period of several months, but return is possible

The Patient's Clinical Examination 
Involuntary motions that might be universal or unilateral and involve the face are characteristic of the neurological condition known as Sydenham chorea. On a test of grip strength, having a "milkmaid grip" indicates intermittent hypotonia.
 Pericardial friction rub, holosystolic murmur of mitral/aortic regurgitation, and very infrequently diastolic; very infrequently signs of heart failure
.
Skin - Subcutaneous nodules (10%): firm, painless protuberances on extensor surfaces involving knees, elbows, wrists, and occiput; more common in severe ARF; lasts for many weeks
– Erythema marginatum is characterized by a pink rash that is evanescent and has pale centers and rounded or serpiginous edges. It can be observed on the trunk and the proximal extremities. Blanches when pressure is applied and can be produced by heat; rarely occurs on the face; normally does not cause pruritis;

The differential diagnosis includes systemic lupus erythematosus, post-streptococcal reactive arthritis, juvenile rheumatoid arthritis, infectious arthritis, myocarditis (viral or idiopathic), innocent cardiac murmur, cardiomyopathy, Tourette syndrome, Kawasaki syndrome, pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS), Lyme disease and  Henoch-Schonlein purpura

Results From the Laboratory 

Initial Examinations (in the lab and with imaging)
When serologic proof of GAS infection cannot be acquired, the diagnosis must be questioned; however, chorea and chronic indolent rheumatic carditis are exceptions to this rule due to their delayed start of symptoms.
- A quick test for streptococcal antigen that has a high probability before the test
- A GAS throat culture that is positive
- An elevated or growing antistreptococcal antibody titer (ASO or ADB); ASO peaks between three and five weeks after an infection, whereas ADB peaks between six and eight weeks after an infection. A growth in titer is preferable to the result of just a single titer.
ESR and CRP are considered to be acute-phase reactants, and their levels are virtually always found to be elevated in ARF.
 Complete blood count with differential: normocytic anemia, leukocytosis
 Pericarditis symptoms on the electrocardiogram, include PR prolongation and AV block
If a substantial effusion or septic arthritis is suspected, aspiration of the joint and examination of the synovial fluid should be performed.
 Valve disease, pericardial effusion, and chamber size and function can be seen on an echocardiogram.
Because of the 18% frequency of subclinical carditis, an echocardiography must to be performed on every instance of confirmed OR suspected ARF within a period of 12 weeks.
Additional Examinations, as well as Other Important Factors
It is helpful to monitor the acute disease process using CRP, which can initially be trended twice weekly, then once every one to two weeks until it reaches normal levels.
 Due to the morbidity associated with untreated cardiac illness, it is recommended that serial echocardiograms be performed on all confirmed instances of ARF (2)[C]. This is the case even if the initial screen was negative for carditis.
It is recommended that throat cultures be performed on everyone living in the home. Antibiotics ought to be taken even if there are no obvious symptoms if the test comes back positive.
The Interpretation of Tests
An earlier course of treatment with aspirin or steroids may produce misleadingly negative laboratory results.

Treatment consists of antibiotics and anti-inflammatory medications (such as aspirin or naproxen). In addition, any other symptoms, such as chorea, dysrhythmia, carditis, or heart failure, should be treated as they arise.

The First Line Of Defense Is Medication

 Eradication: Treatment for a GAS infection should be initiated within nine days of the onset of sickness in order to prevent ARF. If the diagnosis is confirmed, secondary prophylaxis should be started.
 If there is no history of penicillin allergy: - Penicillin VK 250 mg PO BID for 10 days, benzathine penicillin G IM for 1 day, or amoxicillin 50 mg/kg PO for 10 days (aminoxicillin is recommended for use in pediatric patients).
 If you have an allergy to penicillin, try a first-generation cephalosporin taken orally (PO) for 10 days, azithromycin 12 mg/kg taken orally (PO) for 5 days, clindamycin 7 mg/kg/dose taken orally (PO) for 10 days, or clarithromycin 7.5 mg/kg/dose taken orally (PO) for 10 days.
 Arthritis: Given its superior side effect profile and lower incidence of Reye syndrome, naproxen is currently suggested over aspirin as the treatment of choice. The dosage range is 10 to 20 mg/kg/day, split twice daily.
 In cases of carditis in which heart failure, AV block of the third degree, or other severe indications are present, suitable conventional therapy should be commenced as recommended.
Although non-steroidal anti-inflammatory drugs (NSAIDs), glucocorticoids, and intravenous immunoglobulin (IVIG) are not advised for the treatment of carditis, severe carditis associated with abrupt cardiac failure may warrant the use of glucocorticoids.
Chorea is typically self-limiting and does not require treatment in most cases.
In severe cases, valproic acid or carbamazepine may be prescribed as treatment options. Only patients who have failed initial care are eligible to receive IVIG and glucocorticoids. Because of the extrapyramidal adverse effects, antipsychotics are not typically employed in treatment.

Two-Thirds Line

However, the Infectious Diseases Society of America does not recommend using erythromycin in the presence of penicillin allergy. This recommendation comes from the New Zealand Guidelines Group.



A Referral  
When dealing with severe chorea, a pediatric neurologist or movement expert can help guide treatment. The management of atrial fibrillation should require the participation of a cardiologist.

Surgical Methods and Operations 

The bulk of treatment for valve stenosis is surgical repair, as it is a late-stage complication that results from fibrosis and calcification.


Admission 
An initial hospitalization could be helpful for diagnosing the condition and making sure everything is stable.
Those suffering from heart failure must be admitted to the hospital immediately.
 Intravenous fluids should only be given if there are signs of hypotension or dehydration; use extreme caution in cases of heart failure.
 Nursing: Activity to the extent that the patient is able

Follow-Up Antibiotic treatment should begin at the time of diagnosis and continue for at least 21 years or for 5 years after the diagnosis, whichever occurs later. Secondary prophylaxis should begin at this time.
Because a relapse of the disease can make the severity of carditis much worse, indefinite preventive antibiotics may be suggested based on the extent of the damage to the heart.
Injections of long-acting benzathine penicillin G given intramuscularly (IM) once a month are the recommended form of prophylaxis. As an alternative, penicillin V taken orally 250 mg twice daily is also an option.
– If you have an allergy to penicillin, take sulfadiazine at a dosage of 0.5 to 1.0 grams per day. – If you have an allergy to penicillin and sulfa drugs, take azithromycin.
– Clindamycin is the medication that should be administered to penicillin-treated patients who develop GAS pharyngitis while they are on penicillin.
If there is a possibility of ARF but there is still some doubt, the patient should be treated with secondary antibiotic prophylaxis for a full year until they have another echocardiography.
The period of routine antibiotic prophylaxis for dental treatments is no longer suggested for patients who have RHD. However, the duration of prophylaxis for patients who have ARF but no evidence of carditis does not change.
Monitoring of the Patient
After the first diagnosis, monitoring should occur once every week, and then every six months after that, depending on the patient's clinical stability.
Considerations Relating to Children
Caution should be exercised while giving aspirin to youngsters because of the possibility of Reye syndrome.
Things to Think About When Expecting
Could make valve disease worse, in particular mitral stenosis in patients with RHD. Patients who are pregnant should be sent to a cardiologist.

Diet 
There are no dietary limitations, although patients with heart failure should limit their sodium intake.

Forecast or prognosis 
The degree of carditis during an acute attack is directly related to the extent to which long-term complications are confined to the patient's heart.
The first year after a diagnosis is when recurrences are most likely to occur.


Problems and obstacles 
RHD can occur 10 to 20 years after ARF, with mitral more prevalent than aortic regurgitation, and can develop to mitral stenosis. Recurrence of ARF owing to GAS reinfection RHD can occur 10 to 20 years after ARF. The most serious of these complications is heart failure.
Patients who have a history of ARF are at a greater risk of developing infective endocarditis in the years to come. Jaccoud arthropathy is a persistent condition that does not cause any pain to the hands or feet.

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