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Rheumatic fever by dr. Emmanuel rusingiza. My name is emmanuel rusingiza.
I am a pediatric pediatric cardiologist at kigali university teaching hospital this morning. Im going to talk about diagnosis and management or acute rheumatic fever as outlined. We will go through the definition and overview of acute.
Rheumatic fever. The epidemiology. Pathophysiology.
Diagnosis. Investigations. Differential.
Diagnosis and management. Overview. Acute.
Rheumatic fever. Is defined. As a delayed.
Autoimmune. Response. To an untreated.
Group. A streptococcal infection. Mainly affecting the throat acute rheumatic fever may involve the heart.
The joints. The central nervous system and or the skin. The signs and symptoms may include any or all of the following.
Arthritis fever carditis rash sydenhams chorea and subcutaneous nodules group a streptococcal throat infections occur in children throughout the world with peak ages between 5 and 15 years the number of children affected in each region. Varies depending on environmental conditions level of poverty quality and availability of health care over the past century acute. Rheumatic fever.
And rheumatic heart disease have become rare in developed countries. As living conditions have become more hygienic and less crowded with improved nutrition and access to appropriate medical care. Repeated group.
A streptococcal infections and recurrent. Acute rheumatic fever can lead to chronic heart. Valve damage.
That is called rheumatic heart disease. Rheumatic heart disease requires expensive heart valve surgery. If damaged heart valves are not repaired or replaced by major open heart.
Surgery the condition is often fatal. Epidemiology it is estimated that about. 156 million people are affected world wide and among them 24.
Million are children between 5 and 14 years old in developing countries acute rheumatic fever and rheumatic heart disease are the disease of poverty. But they are indicated in industrialized countries since 20th century as thought previously the following factors increase the risk of developing acute. Rheumatic fever.
Overcrowding and poor standards of housing reduced access to health care and living in tropical climates acute rheumatic fever is most common in children between the ages of 5 and 15 years. It is less common after the age of 35 years. And is rare under 4 years and over 40 years of age pathophysiology.
As pathophysiology. Not everyone is susceptible to acute rheumatic fever. And not all.
Group. A streptococcus strains are capable of causing. Acute rheumatic fever in a susceptible host.
It is likely that 3 5. Of people in any population have an inherent susceptibility to acute rheumatic fever. Although the basis of susceptibility is unknown some strains of group.
A streptococcus are called rheumatogenic particularly streptococcal m protein. Although the basis of rheumatogenicity is also unknown. This is a picture of a patient who presents tonsillopharynx infection by group a streptococcus it shows severely inflamed tonsils with presence of pus and the culture has revealed group.
A streptococcus so acute rheumatic fever is sequela of untreated or inadequately. Treated. Group.
A streptococcus infection of the tonsillopharynx studies. Have concluded that there is a molecular mimicry between group a streptococcus antigens and human host tissue. That is believed to be the basis of pathogen.
Host cross reactivity best documented with cardiac proteins such as myosin laminin and vimentin point of clarification in acute rheumatic fever. The patients immune system produces antibodies against the m protein of the group. A streptococcus bacterium.
These antibodies appropriately bind to the antigen on the surface of the bacteria to eradicate the primary infection. But occasionally these same antibodies cross react with the patients own cardiac proteins given the structural similarities between those proteins and the end protein of group. A strep.
This molecular mimicry is believed to be the basis for cardiac pathology related to acute rheumatic fever. And rheumatic heart disease. The patients immune response is initiated after initial exposure to the bacteria.
However there is a latency period of about three weeks before the patient develops symptoms of acute rheumatic fever. This is due to the lag between initial antibody production and the cross reactivity of these antibodies with the patients own tissue proteins at the time of development of acute. Rheumatic fever symptoms.
The host immune system has eradicated the initial group. A strep infection. The progression of the disease is done as following it starts initially by a group a streptococcus throat infection.
Which due to a certain number or factors leads to acute rheumatic fever. And during repetitive episodes of group. A streptococcus infection in the future.
It causes recurrent. Acute rheumatic fever. That leads to rheumatic heart disease.
With all its complications diagnosis. The diagnosis of acute rheumatic fever remains a clinical decision. Its the original specific laboratory test.
It is known that overdiagnosis of acute rheumatic fever will lead to unnecessary treatment over a long time while underdiagnosis leads to further attacks of acute rheumatic fever cardiac damage and premature death. The diagnosis of acute rheumatic fever is usually guided by jones criteria developed in 1944. And adopted most recently by the world health organization.
The jones criteria include major criteria and minor manifestations. Plus. Evidence of preceding group.
A streptococcus infection. This table summarizes. The jones criteria.
And the first column shows the major manifestations that include arthritis carditis subcutaneous nodules erythema marginatum and sydenhams chorea. The column in the middle shows. Minor manifestations.
Which are fever arthralgia prolonged pr interval on ecg and raised ecr or crp. The evidence of recent group.
A streptococcus infection. Include the positive culture of the throat swab. The raised anti streptolysin o titer and the raised anti dnase b.
Arthritis is the common symptom. And it is characterized by pain redness and swelling in the joints and it affects commonly the big joints like the ankles the knees. The wrists the elbow and less commonly the small joints.
It is often the first complaint and arthritis is usually migratory disappearing in one joint as it begins in another the carditis. Which is defined as an inflammation of the heart is commonly present as a heart murmur chest pain and or difficulty breathing may be present in severe cases. Less commonly people with acute rheumatic fever.
Present with subcutaneous nodules and erythema marginatum with specific characteristics. Subcutaneous nodules. Are painless lumps seen on the outside surfaces of major joints.
They are often present for about one to two weeks duration and are more commonly present. When the patient also has evidence of carditits erythema marginatum starts out as painless flat pink patches on the skin that spread outward in a circular pattern. This is often an early symptom of acute rheumatic fever and often spares the face this rash may be present for months.
After the onset of acute rheumatic fever sydenhams chorea is a twitching jerking movements and muscle weakness. Most obvious in the face hands and feet. It is more common in teenagers and females.
It may begin up to three to four months after the streptococcal infection. It may appear on both sides or only one side of the body and often appears without other symptoms point of clarification. The mean duration of chorea is documented in the literature as 12 15 weeks.
But please note that some episodes may persist for as long as 6 12. Months. The fever is defined as a core temperature greater than 38 degrees.
And it can go up to high values. The evidence of group. A streptococcus infection is required to confirm a case of acute rheumatic fever with the above signs and symptoms group.
A streptococcus may not be found on a throat swab since the infection may be resolved at the time of onset of acute. Rheumatic fever. Symptoms.
Serum anti streptolysin. O. Titer reaches the peak level.
Around three to six weeks after infection and starts to fall at six to eight weeks serum anti dnase b reaches a peak level up to six to eight weeks after infection and starts to fall at around three months after the infection. The first episode of acute rheumatic fever can be confirmed if there are two major criteria or one major criteria and two minor manifestations. Plus.
An evidence of preceding group. A streptococcus infection. Recurrent acute rheumatic fever.
Without rheumatic heart disease. Can be confirmed. As the previous first episode.
The recurrent acute rheumatic fever with existing rheumatic heart disease can be confirmed. If there are two minor manifestations. Plus evidence of preceding group a streptococcus infection.
However different regions have slightly modified guidelines to assist clinicians with local variations in acute rheumatic fever presentation in this regard. The involvement of only one joint also called monoarthritis polyarthralgia in children. Who are at high risk of acute rheumatic fever and subclinical carditis.
Proved by echocardiogram have been proposed to be among the major criteria. The differential diagnosis is made with septic arthritis connective tissue valvular arthropathy. Sickle cell.
Anemia mitral valve prolapse infective endocarditis and many other diseases. Which present the similar clinical manifestations like acute rheumatic fever investigations. The investigations should be fbc esr crp blood cultures.
If febrile especially for the differential diagnosis with infective endocarditis. The immunologic markers of group a streptococcus infection. Which are aso and anti dnase b.
Throat swab ekg chest x. Ray. If there is an evidence of colitis and echocardiogram this echocardiography image shows a severely damaged mitral valve.
Which is thickened look at the posterior leaflet. Which is also retracted and during systole. There is a very bad coaptation of the mitral leaflets that results in massive mitral regurgitaiton and dilation of the left atrium.
This patient was admitted for severe heart failure due to rheumatic heart disease management. The treatment of the acute illness. Includes benzathine.
Penicillin g. Single. Injection or oral penicillin for 10 days.
And in case of allergy erythromycin. Is indicated relief of symptoms and signs with non steroid anti inflammatory drugs especially aspirin or corticosteroids carbamazepine and valproic acid can be given for severe cases of sydenhams chorea. The management of acute rheumatic fever should be based on the following principles admission for acute diagnosis receive clinical care and education about preventing further episodes of acute rheumatic fever.
Initial echocardiogram is very important to identify and measure. The heart. Valve damage.
Long term. Preventive management. Should be organized before this discharge.
The long term management. Includes regular secondary prophylaxis. Regular medical review.
Regular. Dental review. Echocardiogram.
Following each episode of acute rheumatic fever. And routine echocardiogram secondary prophylaxis should be done by benzathine penicillin. G.
Im every three to four weeks and the standard dose is 12. Million units for patients who weigh. 30.
Kgs or greater and the half dose of 600000 units for patients who are under 30 kgs penicillin v. Can be used if benzathine penicillin injections are not tolerated or injections are contraindicated the standard dose is one tab of 250 milligrams oral twice daily here i would like to insist on the necessity to give the injectable form of penicillin because it has shown better results compared to the oral form of penicillin erythromycin is given if there is proven allergy to penicillin the standard dose is 250 milligrams oral twice daily the duration of secondary prophylaxis should be done as following when acute rheumatic fever is identified. Without proven carditis.
The minimum duration should be five years after the last episode of acute rheumatic fever. Or until 18 years. For the mild to moderate forms of rheumatic heart disease.
The minimum duration should be 10 years after the last. Acute rheumatic fever. Or until the age of 25 years for severe.
Rheumatic heart disease. And following cardiac surgery for rheumatic heart disease. Patients should continue medication for life.
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