Rheumatic fever is an inflammatory, non-suppurative complication that might occur two to four weeks following Group A Streptococcus pharyngitis in an acute form. It is an autoimmune response to an earlier infection.
Although it can occur at any age, it is most commonly seen in children aged 5–15. Most of the cases are seen in low-income countries and those with minimal access to healthcare systems. This might be due to household overcrowding or the misuse of antibiotics.
The mean incidence of acute rheumatoid fever is 19/100,000 school-aged children worldwide, while in the United States and other high-income countries, the mean incidence is lower: <= 2/100,000 school-aged children.
The pathophysiology of the autoresponse to rheumatic fever is thought to be due to molecular mimicry. In this case, the similarity between the antigens of the invading pathogen and the antibodies of the host leads to the activation of T and B cells inside the body. However, due to this molecular mimicry, the antibodies and the antigens cross-react inside the body, resulting in the clinical features of rheumatic fever.
The clinical manifestations of acute rheumatic fever are divided into major manifestations and minor manifestations.
The five major manifestations are:
It is considered the earliest manifestation of acute rheumatic fever, which happens within a few weeks of being infected. Arthritis involves different joints of the body, including the knees, wrists, elbows, and ankles, with the leg joints being affected initially. The general presentation is frequently more subjective and described as joint pain rather than objective inflammation. The pain is usually "migratory," moving from one joint to the other (polyarthritis).
Once the diagnosis is done, early treatment with non-steroidal anti-inflammatory drugs or corticosteroids has a positive impact on joint symptoms. If symptoms do not improve within 48 hours of initiating therapy, the diagnosis should be reconsidered.
One disadvantage of introducing NSAIDs earlier before other signs and symptoms of rheumatic fever occur is that patients might appear to have monoarthritis rather than polyarthritis, which will eventually lead to misdiagnosis. Because of this limitation, NSAIDs are better delayed if acute rheumatic fever is suspected and only one joint is affected, and they can be initiated thereafter once the diagnosis of acute rheumatic fever is confirmed. Meanwhile, paracetamol can be used to control joint pain.
Carditis associated with acute rheumatic fever occurs within 3 weeks of a Group A Streptococcus infection. It involves the whole heart (pericardium, epicardium, myocardium, and endocardium), with endocarditis being the most common manifestation of acute rheumatic fever.
Endocarditis usually presents with the most typical form of valvulitis: mitral regurgitation.
Subclinical carditis might happen, but it can be detected by echocardiography only because, unlike clinical carditis, it cannot be detected clinically due to a lack of auscultatory findings.
Is a manifestation that occurs one to six months after being infected with Group A streptococci, making it the most delayed clinical manifestation. It is a neurological disorder that is characterized by involuntary muscle movements and weakness of the face, legs, and hands. A physical examination is done to confirm muscle weakness by simply asking the patient to squeeze the examiner’s fingers. Patients with Sydenham's chorea will not be able to squeeze firmly and constantly; rather, they will squeeze and release in a phenomenon known as the “Milkmaid sign”.
Children usually self-isolate and develop emotional changes; some patients develop obsessive-compulsive disorder patterns and rarely psychosis.
Sydenham chorea is commonly recurrent and might reoccur during pregnancy and when using contraceptives due to hormonal changes.
It occurs early in the course, but sometimes it might be noticed during recovery. It is characterized by an evanescent, pink, or faintly red nonpruritic rash that mainly involves the trunk and sometimes the limbs with no facial involvement; they are very recognizable after a hot bath.
It rarely appears alone and is usually seen with other manifestations, especially carditis.
They occur a few weeks after infection. They are firm, symmetric, well-seated, painless bumps that are small in diameter (ranging from a few millimeters to 2 cm) and are located near tendons or over a bony area, especially the elbow area. Like Erythema marginatum, subcutaneous nodules are rarely seen as a sole manifestation.
The Jones Criteria are used to diagnose acute rheumatic fever and typically include major and minor criteria. Here's a summary:
These criteria are used by healthcare professionals to assist in the diagnosis of acute rheumatic fever, and they help ensure that the diagnosis is accurate and appropriate for the patient's clinical presentation. However, it's important to note that diagnosis and management should be carried out by healthcare professionals with expertise in this area.
Primary prevention involves administering antibiotics, such as penicillin, promptly to individuals diagnosed with Group A streptococcal pharyngitis (strep throat) to prevent the development of acute rheumatic fever.
After an initial episode of acute rheumatic fever, prescribe extended antibiotic therapy, typically penicillin, to prevent recurrent episodes and the associated risk of rheumatic heart disease. The choice of antibiotic and duration depends on the patient's specific clinical situation.
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