Rheumatic Fever Overview and Diagnosis
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Questions and Answers

What is one circumstance where the diagnosis of acute RF can be made without strict adherence to the Jones Criteria?

  • When the patient shows signs of arthritis.
  • When chorea occurs as the only major manifestation. (correct)
  • When fever is present as a major symptom.
  • When cardiac surgery has been previously performed.

    • What percentage of patients with acute RF typically experiences migratory polyarthritis?

  • 50%
  • 75% (correct)
  • 85%
  • 90%

    • Which joints are most commonly involved in rheumatoid arthritis associated with acute RF?

  • Only shoulder joints.
  • Hips and small joints of the feet.
  • Knees, ankles, wrists, and elbows. (correct)
  • Spinal joints and small joints of the hands.

    • What characterizes the joint involvement in migratory polyarthritis associated with acute RF?

    <p>Affected joints can return to normal within days.</p> Signup and view all the answers

    Which feature of rheumatic carditis is universally present?

    <p>Endocarditis (valvulitis).</p> Signup and view all the answers

    What are the main manifestations of rheumatic carditis?

    <p>Pancarditis with myocarditis, pericarditis, and endocarditis.</p> Signup and view all the answers

    What percentage of patients with acute RF experience Sydenham Chorea?

    <p>10-15%</p> Signup and view all the answers

    What clinical evidence is rarely associated with myocarditis in rheumatic carditis?

    <p>Endocarditis.</p> Signup and view all the answers

    What is the primary prevention method for acute rheumatic fever (RF)?

    <p>Appropriate antibiotic therapy before the 9th day of symptoms</p> Signup and view all the answers

    What is the regimen of choice for secondary prevention of RF?

    <p>Single IM injection of benzathine penicillin G every 4 weeks</p> Signup and view all the answers

    In the case of a patient allergic to penicillin, which antibiotic can be used for prophylaxis?

    <p>Clarithromycin</p> Signup and view all the answers

    What is a hallmark symptom of Henoch-Schonlein Purpura (HSP)?

    <p>Skin rash</p> Signup and view all the answers

    At what age does HSP most commonly peak?

    <p>3-10 years</p> Signup and view all the answers

    Which demographic group has the highest incidence of HSP?

    <p>White and Asian children</p> Signup and view all the answers

    HSP is primarily mediated by which Ig class?

    <p>IgA</p> Signup and view all the answers

    What is the estimated incidence of HSP per year in children?

    <p>14-20 per 100,000</p> Signup and view all the answers

    What is the most common etiology of rheumatic fever?

    <p>Group A Streptococcus upper respiratory tract infections</p> Signup and view all the answers

    Which age group has the highest incidence of acute rheumatic fever?

    <p>Children aged 5-15 years</p> Signup and view all the answers

    Which is NOT one of the major criteria used for diagnosing acute rheumatic fever?

    <p>Chest Pain</p> Signup and view all the answers

    What is required for the diagnosis of acute rheumatic fever alongside the Jones Criteria?

    <p>Evidence of preceding Group A Streptococcus infection</p> Signup and view all the answers

    Which minor criterion is included in the diagnosis of acute rheumatic fever?

    <p>Elevated acute phase reactants</p> Signup and view all the answers

    Which Serotype of Group A Streptococcus is frequently associated with acute rheumatic fever?

    <p>M type 1</p> Signup and view all the answers

    How long after a GAS pharyngitis does acute rheumatic fever typically onset?

    <p>2-4 weeks</p> Signup and view all the answers

    What is the primary reason for high rates of rheumatic heart disease in developing countries?

    <p>High annual incidence of acute rheumatic fever</p> Signup and view all the answers

    What symptom is least likely to be associated with chorea?

    <p>Permanent neurologic sequelae</p> Signup and view all the answers

    What is the characteristic rash of acute rheumatic fever known as?

    <p>Erythema marginatum</p> Signup and view all the answers

    Which treatment is NOT recommended for patients with acute rheumatic fever?

    <p>Immediate elective surgery</p> Signup and view all the answers

    What are subcutaneous nodules associated with in acute rheumatic fever?

    <p>Indicating significant rheumatic heart disease</p> Signup and view all the answers

    What is the primary purpose of antibiotic therapy in acute rheumatic fever?

    <p>To eradicate GAS from the upper respiratory tract</p> Signup and view all the answers

    Which of the following is a clinical maneuver used to elicit features of chorea?

    <p>Darting tongue examination</p> Signup and view all the answers

    What medication should be administered to a penicillin-allergic patient with acute rheumatic fever?

    <p>5 days of azithromycin</p> Signup and view all the answers

    In which condition should corticosteroids be administered as part of the treatment for acute rheumatic fever?

    <p>Patients with carditis and cardiomegaly</p> Signup and view all the answers

    What is the progression of skin lesions in this condition?

    <p>Pinkish maculopapules to petechiae or purpura</p> Signup and view all the answers

    Which joint is primarily affected with arthritis in this condition?

    <p>Knees and ankles</p> Signup and view all the answers

    What is the definitive test for diagnosing this condition?

    <p>Skin biopsy</p> Signup and view all the answers

    What is a common gastrointestinal complication of this condition?

    <p>Bowel infarction</p> Signup and view all the answers

    What is the typical duration of this condition?

    <p>4 weeks</p> Signup and view all the answers

    Which of the following is considered a manifestation during active disease?

    <p>Liver enlargement</p> Signup and view all the answers

    What percentage of children experience recurrences of this condition?

    <p>15-60%</p> Signup and view all the answers

    What treatment is indicated for life-threatening manifestations?

    <p>Corticosteroids</p> Signup and view all the answers

    Study Notes

    Rheumatic Fever

    • Caused by Group A Streptococcus (GAS) upper respiratory tract infections
    • Annual incidence exceeds 50 per 100,000 children in developing countries
    • Most common form of acquired heart disease worldwide
    • Two-thirds of patients with acute rheumatic fever have a history of upper respiratory tract infection
    • Not all GAS serotypes cause rheumatic fever. Serotypes 1, 3, 5, 6, 18, and 29 are frequently isolated from patients
    • Incidence peaks in children 5-15 years old
    • Onset of rheumatic fever occurs approximately 2-4 weeks after GAS pharyngitis

    Diagnosis

    • Diagnosis requires evidence of preceding GAS infection
    • Requires either 2 major or 1 major and 2 minor criteria
    • Major criteria:
      • Migratory polyarthritis
      • Carditis
      • Sydenham chorea
      • Erythema marginatum
      • Subcutaneous nodules
    • Minor criteria:
      • Arthralgia (only if arthritis is not a major criterion)
      • Fever
      • Elevated acute phase reactants (ESR, C-Reactive protein)
      • Prolonged P-R interval on ECG (unless carditis is a major criterion)
    • Diagnosis can be made without strict adherence to the Jones Criteria in certain circumstances

    Clinical Manifestations

    Migratory Polyarthritis

    • Affects approximately 75% of patients
    • Typically involves larger joints (knees, ankles, wrists, elbows)
    • Joints are hot, red, swollen, and tender
    • Arthritis is rarely deforming
    • Migratory in nature; inflammation in one joint resolves in 1-3 days, but another joint may become inflamed
    • Dramatic response to low-dose salicylates

    Carditis

    • Occurs in 50-60% of cases
    • Most serious manifestation of rheumatic fever
    • Characterized by pancarditis (inflammation of myocardium, pericardium, and endocardium)
    • Endocarditis is universal, but pericarditis or myocarditis are variable
    • Presents with tachycardia, cardiac murmurs, cardiomegaly, heart failure, hepatomegaly, peripheral and pulmonary edema

    Sydenham Chorea

    • Occurs in 10-15% of patients
    • Latent period of months after GAS infection
    • Presents as a movement disorder (motional liability, incoordination, uncontrollable movements, facial grimacing)
    • Exacerbated by stress, disappears with sleep
    • Rarely causes permanent neurologic sequelae
    • Clinical maneuvers include milkmaid’s grip, pronator test, darting tongue, and handwriting evaluation

    Erythema Marginatum

    • Rare manifestation (approximately 1%)
    • Erythematous, serpiginous, macular lesions with pale centers
    • Not pruritic
    • Occurs primarily on the trunk and extremities, not the face
    • Accentuated by warming the skin

    Subcutaneous Nodules

    • Rare (≤1% of patients)
    • Firm nodules approximately 1 cm in diameter
    • Occur along extensor surfaces of tendons near bony prominences
    • Correlated with significant rheumatic heart disease

    Treatment

    • Bed rest and monitoring for evidence of carditis
    • Antibiotic Therapy:
      • 10 days of oral penicillin, amoxicillin, erythromycin, or a single IM injection of benzathine penicillin
      • If penicillin allergic: 10 days of erythromycin, 5 days of azithromycin, or 10 days of clindamycin
      • Long-term antibiotic prophylaxis is required for secondary prevention
    • Anti-inflammatory Therapy:
      • Salicylates for patients with polyarthritis and carditis without cardiomegaly or CHF
      • Corticosteroids for patients with carditis with cardiomegaly and/or CHF
    • Supportive therapies for moderate to severe carditis: digoxin, fluid and salt restriction, diuretics, oxygen
    • Sydenham Chorea: Phenobarbital, haloperidol, chlorpromazine may be helpful. Some patients benefit from corticosteroids

    Prevention

    Primary Prevention

    • Appropriate antibiotic therapy before the 9th day of acute GAS pharyngitis is highly effective in preventing initial attacks

    Secondary Prevention

    • Continuous antibiotic prophylaxis is required for patients who have already suffered an attack of acute RF
    • Prophylaxis continues until age 21 or 5 years since the last attack, whichever is longer
    • Lifelong prophylaxis may be necessary for patients with carditis and residual heart disease
    • Regimen of choice: Single IM injection of benzathine penicillin G every 4 weeks
    • Oral Penicillin V 250 mg twice daily is equally effective
    • Macrolides may be used for patients allergic to penicillin

    Henoch-Schonlein Purpura (HSP)

    • Common vasculitis of small vessels
    • Most common cause of non-thrombocytopenic purpura in children
    • Affects all ethnic groups but is more common in white and Asian populations
    • Incidence is estimated at 14-20 per 100,000 children
    • Affects males more than females
    • Etiology is unknown but may follow URTI, especially GAS
    • Pathology is IgA-mediated vasculitis with complement activation

    Clinical Manifestations

    • Peak age is 3-10 years
    • May be acute or insidious with sequential occurrence of symptoms over weeks or months
    • Skin rash: Hallmark symptom, present in all cases. Maculopapules progress to petechiae or purpura which is palpable and not blanchable. Lesions are typically symmetric and occur in gravity-dependent areas.
    • GIT involvement: Occurs in up to 80% of patients, due to edema and damage of blood vessels, causing intermittent colicky abdominal pain and GIT bleeding.
    • Arthritis: Affects approximately 75% of patients, mainly involving knees and ankles. Usually resolves within 2 weeks without residual deformity.
    • Renal involvement: Occurs in up to 50% of patients, including nephritis, nephrosis, or acute renal failure.
    • Other manifestations: Low-grade fever, fatigability, hepatosplenomegaly, lymphadenopathy.

    Diagnosis

    • Usually clinical, but definitive test is skin biopsy showing leukocytoclastic angiitis.

    Complications

    • GIT: Intussusception, bowel infarction, perforation
    • Neurological involvement: Seizures, paresis, coma
    • Other: Cardiac and eye involvement, pancreatitis, pulmonary or intramuscular hemorrhage, testicular torsion

    Treatment

    • Supportive therapy: Hydration, nutrition, analgesia.
    • Corticosteroids may be indicated for GIT and other life-threatening manifestations, but do not alter overall prognosis or prevent renal disease.

    Prognosis

    • Self-limited disease lasting approximately 4 weeks
    • Excellent overall prognosis, but death is possible due to complications
    • 15-60% of children experience recurrences, typically within 4-6 months
    • Relapses are usually milder than initial presentation
    • Renal involvement at presentation may lead to chronic renal disease or hypertension. Follow-up for 6 months with urinalysis and blood pressure measurement is recommended.

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    Description

    Explore the causes, incidence, and diagnostic criteria of rheumatic fever in this quiz. Understand the relationship between Group A Streptococcus infections and the development of this prevalent heart disease in children. Test your knowledge of both major and minor diagnostic criteria.

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