Rheumatic Fever: Symptoms, Diagnosis, and Treatment

Rheumatic Fever: Symptoms, Diagnosis, and Treatment

Rheumatic fever is an autoimmune disease, common in children, that occurs after a Streptococcus infection. The disease causes damage to many organs such as the heart, joints, nervous system, and skin.

Clinical Manifestations:

• Arthritis:

• Arthritis in rheumatic fever:

• Inflammation of large joints (knees, ankles, elbows, wrists): swelling, warmth, redness, noticeable pain, limited mobility.

• Characteristics: Migratory (moving from one joint to another), self-healing after 1 week, no sequelae.

• Cardiac Inflammation:

• Pericarditis: Pericardial effusion.

• Myocarditis: Inflammation of the heart muscle.

• Valvular Heart Disease: Heart murmur due to valve regurgitation or stenosis.

• Pancarditis: Inflammation of all heart structures.

• General Clinical Symptoms:

• Chest pain, palpitations, fatigue, shortness of breath.

• Heart murmur at the apex of the heart.

• Sydenham’s Chorea:

• Manifestations:

• Sudden personality changes (poor academic performance, irritability, rambling speech).

• Loss of fine motor coordination (writing, using chopsticks).

• Examination: Abnormal movements spreading from the fingertips to the limb roots, trunk, involuntary, decisive, disappears during sleep, increases with excitement, etc…

• Rash:

• Characteristics: Red, serpentine or circular, pale center, not itchy, disappears after a few days, only present on the back, limb roots, not on the face.

• Subcutaneous Nodules:

• Manifestations: Subcutaneous nodules around the joints, 0.2-2cm in diameter, painless, movable, disappear after a few days.

Diagnosis:

• Initial diagnosis according to the Jones Criteria 1992:

• 2 major criteria OR (1 major criteria + 2 minor criteria) AND evidence of streptococcal infection.

• Cases not requiring evidence of streptococcal infection:

• Sydenham’s chorea

• Silent cardiac inflammation.

• Cases not requiring complete Jones Criteria 1992:

• Recurrent rheumatic fever: requires 1 major criteria/ 2 minor criteria + evidence of streptococcal infection + history of rheumatic fever.

Diagnosis of Sydenham’s Chorea:

• History of chorea.

• Exclusion of other diseases:

• Huntington’s chorea.

• Wilson’s disease.

• Extrapyramidal disorders in the sequelae of central nervous system damage (history of encephalitis, meningitis).

• Dissociative disorders.

Silent Cardiac Inflammation:

• 3 signs:

• Sick for over 1 month (fatigue, loss of appetite,…)

• Clinical signs of heart failure.

• Physical heart murmur at the apex of the heart.

• Exclude:

• Infective endocarditis (children with fever).

• Severe anemia.

Jones Criteria 1992 for Diagnosis:

• Major Criteria:

• Cardiac inflammation

• Polyarthritis

• Chorea

• Ring rash

• Subcutaneous nodules

• Minor Criteria:

• Fever

• Joint pain

• Prolonged PR interval on EKG

• Elevated erythrocyte sedimentation rate

• Elevated CRP

Jones Criteria 2015:

• Differences from Jones 1992:

• Added cardiac inflammation on echocardiogram to diagnostic criteria: Clinical cardiac inflammation AND/OR echocardiogram.

• Low/high risk group: One joint or multiple joint inflammation or multiple joint pain.

Evidence of Streptococcal Infection:

• Jones 1992:

• Throat culture: Group A Streptococcus.

• Rapid throat swab test (+) with LCK (or recently had scarlet fever).

• Elevated ASLO.

Jones Criteria 2015 Minor Criteria:

• Low Risk Group:

• Fever >= 38.5 degrees C.

• Multiple joint pain.

• Erythrocyte sedimentation rate >= 60mm/h first hour.

• Elevated CRP.

• Prolonged PR interval on EKG.

• Medium/High Risk Group:

• Fever >= 38 degrees C.

• One joint pain.

• Erythrocyte sedimentation rate >= 30mm/h first hour.

Evidence of Streptococcal Infection according to Jones 2015:

• Throat culture: Group A Streptococcus + Rapid throat swab test (+) with LCK.

• Elevated ASLO.

Diagnosis of Recurrent Rheumatic Fever according to Jones 2015:

• Differences from Jones 1992:

• 2 major criteria/ 1 major criteria + 2 minor criteria/ 3 minor criteria (medium/high group) + evidence of streptococcal infection.

Low/Medium/High Risk Group:

• Low Risk Group:

• <= 2/100,000 school-aged children per year or

• All ages <= 1/1000 population

• Medium/High Risk Group:

• > 2/100,000 school-aged children per year or

• All ages > 1/1000 population

Treatment:

• Anti-infective

• Anti-inflammatory

• Anti-heart failure

• Anti-convulsant

Anti-infective Treatment:

• Penicillin G: 1 million units/day.

• Penicillin allergy: Erythromycin 1 gram/day x 10 days.

• Secondary prevention: Erythromycin 10 grams/day x 10 days.

Anti-inflammatory Treatment:

• Without cardiac inflammation: Aspirin.

• With cardiac inflammation: Prednisolone.

Aspirin Dosage:

• 100mg/kg/day x 7 days.

• Then reduce to 60mg/kg/day x 3-4 weeks.

• Divide the dose into 4-6 times a day.

• Take with milk, after meals to reduce stomach irritation.

Prednisolone Dosage:

• 2mg/kg/day x 2-3 days.

• Monitor clinical signs, erythrocyte sedimentation rate, CRP, gradually reduce the dose until the end of the treatment (4-8 weeks).

• Combine with Aspirin in the last week of corticosteroid therapy to prevent recurrence, dose 60mg/kg/day x 3-5 weeks.

Rest:

• Without cardiac inflammation: 2 weeks.

• With cardiac inflammation: 4 weeks.

• With heart failure: All the time while heart failure persists.

Prevention:

• Primary prevention with medication (early treatment of streptococcal pharyngitis):

• Penicillin V: 1 million units/day divided into 4 doses x 10 days.

• Oral Erythromycin: 40mg/kg/day divided into 4 doses (children <30kg) x 10 days.

• Benzathine PNC intramuscular injection 1 single dose (>30kg 1.2 million units, <30kg 900,000 units).

• Secondary prevention to prevent recurrence of rheumatic fever:

• Benzathine PNC intramuscular injection 1 dose/ 3-4 weeks (>30kg 1.2 million units, <30kg 900,000 units).

• Oral Penicillin V daily 250mg x 2/day or 400,000 units/day.

• Oral Erythromycin daily 250mg x 2/day.

Duration of secondary prevention:

• Without cardiac inflammation: At least 5 years after the last episode or until 18 years of age.

• First cardiac inflammation: Until 25 years of age or more.

• Chronic valvular heart disease due to rheumatic fever: Lifelong.

• Rheumatic vascular disease: Lifelong.

The latest damage in rheumatic fever: Brain.

Note:

• This article is for informational purposes only and does not replace the advice of a physician.

• If you suspect that you or your child may have rheumatic fever, please see a doctor for prompt diagnosis and treatment.