Rheumatic fever – The aftermath of strep throat

Rheumatic fever – The aftermath of strep throat

Rheumatic fever

Acute rheumatic fever (ARF) is an inflammatory autoimmune response that portrays as a sequela of Group A Streptococci (GAS) infection. If untreated at the first episode, it may robustly precipitate rheumatic heart disease (RHD) and a range of other relentless complications.

Sore throat infection (pharyngitis) may impact anybody, yet special care needs to be taken when it comes to children. Rheumatic fever predominates itself essentially in age group of 5-15 years and is rare in children below 3 years of age and adults. Acute rheumatic fever culminates usually two to three weeks subsequent to pharyngitis, specifically caused by GAS bacteria. Overcrowded living conditions and heredity are known risk factors. At present, developing countries like Eastern Europe, the Middle East, Asia, and some indigenous populations from Australia and New Zealand are at target line to face rheumatic fever and its complications. However, due to proper hygienic standards and routine use of antibiotics to fight the infection, ARF has ceased in developed countries (<10/100,000).

Overall, worldwide prevalence of ARF is 19/100,000; ARF and RHD affect around 20 million people and are way ahead in causing death due to heart failure.

Know the signs and symptoms of ARF
Your child may be suffering from ARF if he/she experiences any of the following major manifestations:

Fever and abdominal pain are obscure symptoms that arrive early.

Polyarthritis is the commonest and first symptom seen in 70% of children accompanied by fever and routinely lasts for 2-4 weeks. Aching and tenderness of joints; seen as swollen, red, and warm. The affected joints are ankles, knees, elbows, and wrists, although shoulders, hips, and small joints of hands and feet may also get involved. The pain switches from one joint to another hence it is referred as migratory pain.

Carditis: Inflammation of the heart tissues (pericardium, myocardium, and endocardium) ensues in 40% people presenting as chest pain, shortness of breath, and high fever. Heart inflammation usually subsides within 5 months. However, it may permanently damage the cardiac valves resulting in aortic valve disease, mitral stenosis, mitral regurgitation, and congestive heart failure. Valve damage produces the characteristic heart murmurs.

Subcutaneous nodules or skin lumps that are firm and painless occur on the extensor surfaces of the elbows, knees, ankles, knuckles, and scalp.

Erythema marginatum displays as painless, non-itchy, raised, pink rings on the trunk and inner surface of the limbs sparing the face. Skin symptoms arise for a short duration and settle down within a day.

Sydenham’s chorea: Presenting lately in 10-30% children, it is preceded by inappropriate laughing and crying and involves rapid and irregular jerking movements of extremities and face. In addition, fluctuating grip strength, darting of tongue, facial grimacing, and explosive speech with or without tongue clucking are important to consider.

Diagnostic tests to be performed are:

Testing for GAS: Recent history of GAS infection or scarlet fever, positive throat culture, increased antistreptolysin O (ASO) titer, positive rapid GAS antigen test, or elevated anti-DNase B antibody levels are absolute indicators for ARF.

Abnormal electrocardiogram (ECG) most often indicates prolonged PR interval and abnormal heart rhythms.

Blood tests: The definite markers that suggest inflammation are high white blood cell count, i.e., erythrocyte sedimentation rate (ESR >120 mm/hr) and C-reactive protein (CRP >2 mg/dL) levels.

Treatment options are:

  1. Bed rest is advised until infection and inflammation are gone.
  2. Intramuscular injection of penicillin is recommended to eradicate the bacteria as well as to prevent come back.
  3. Aspirin and naproxen are given to relieve painful inflammation of joints.
  4. If heart failure happens, prompt delivery of drugs including diuretics, angiotensin converting enzyme inhibitors, and digoxin or even surgery may be required.
  5. Chorea often is self-limiting; however, it may need to be treated with diazepam.

Can ARF be prevented?
Yes, there are two types of prevention strategies:

Primary prevention: Since GAS infection is the primary cause of ARF, proper diagnosis and prompt antibiotic treatment may prevent ARF in most cases.

Secondary prevention: Recurrent ARF is the prime factor for RHD and other complications. Secondary prophylaxis with penicillin is intended for averting recurrence even after throat culture is negative for GAS bacteria. If the patient has ARF with carditis and RHD; then he/she should receive penicillin for 10 years or until the age of 40 years. Whereas a patient with or without carditis and no RHD would have to take penicillin until the age of 21 years.

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