II. Epidemiology

  1. Incidence: 20 million cases/year in developing world
  2. Attack rate: 0.3 to 3% of untreated Streptococcal Pharyngitis cases
  3. Peak age of onset: 6 to 12 years old (rare under age 3 years)

III. Pathophysiology

  1. Follows Streptococcal Pharyngitis by 2-6 weeks (typically 10-28 days)
  2. Autoimmune reaction to Group A Stretococcal Infection
    1. Streptococcal Pharyngitis
    2. Scarlet Fever
    3. Impetigo is NOT associated with Rheumatic Fever

IV. Diagnosis: Jones Criteria

  1. Diagnostic criteria
    1. Two Major Criteria or
    2. One Major and 2 Minor Criteria
  2. Major Criteria
    1. Mnemonic: Jones
      1. J - Joints (Migratory Arthritis)
      2. O - Shape of Heart (Carditis)
      3. N - Nodules (Subcutaneous)
      4. E - Erythema Marginatum
      5. S - Sydenham's Chorea
    2. Carditis
      1. Present in 50-60% of cases
      2. Endocarditis (mitral valve most commonly affected)
        1. Results in Rheumatic Heart Disease
        2. Manifests as valve disease in 10-20 years after carditis in 50%
        3. Mitral Stenosis is most common outcome
      3. Pericarditis
      4. Myocarditis (rare)
    3. Migratory polyarthritis
      1. Most common symptom (occurs in 49-78% of patients)
      2. Significant response to Aspirin or NSAIDS
      3. Migratory Arthritis
      4. Large joints starting with legs and moving to arms
        1. Knees and ankles
        2. Wrists and elbows
        3. Rarely affects spine
      5. Transient (3 days per joint, and 3 weeks total)
        1. Typical course is <1 week
      6. More severe in teens and young adults
    4. Sydenham's Chorea
      1. Abrupt onset of choreoform movements (purposeless, nonrhythmic, involuntary) only present while awake
      2. Motor weakness
      3. Emotional disturbance (outbursts, Psychosis)
      4. Late finding (may occur up to 8 months after initial infection)
      5. Resolves over 2-3 years
    5. Erythema Marginatum (associated with carditis)
      1. Non-pruritic pink to slightly red rings
      2. Involves trunk and proximal extremities, but spares face
      3. Transiently appears, rapidly advances and disappears over months
    6. Subcutaneous Nodules (associated with carditis)
      1. Small, firm painless Nodules on extensor surfaces (esp. dorsal wrist, elbow, anterior knee)
      2. Develop over bony prominences or over tendons
  3. Minor Criteria
    1. Arthralgias
    2. Fever
    3. Elevated Sedimentation Rate (ESR)
    4. Elevated C-Reactive Protein
    5. Prolonged PR Interval on Electrocardiogram
  4. Supporting criteria
    1. Group A Streptococcal Infection precedes episode
      1. Positive GAS Rapid Antigen test or Throat Culture OR
      2. Anti-streptococcal Antibody level increased

V. Differential Diagnosis: Migratory polyarthritis with cardiac involvement, rash

VI. Labs

  1. Complete Blood Count
    1. Variable Leukocytosis
  2. Acute phase reactants markedly increased
    1. Erythrocyte Sedimentation Rate
    2. C-Reactive Protein
  3. Group A Beta Hemolytic Streptococcus testing
    1. Rapid Antigen test with reflex to Throat Culture
    2. ASO Titer
      1. Increased over baseline

IX. Course

  1. Onset 10-28 days after Streptococcal Pharyngitis onset

X. Management

  1. Admit for definitive diagnosis and evaluation
  2. Polyarthritis
    1. Aspirin
      1. Adult: 4-8 g/day
      2. Child: 80-100 mg/kg/day
        1. Consider Naproxen instead due to risk of Reye's Syndrome
    2. NSAIDs
      1. Consider if Aspirin cannot be used
  3. Carditis
    1. Corticosteroids as directed by cardiology
    2. Congestive Heart Failure management
  4. Treat Streptococcal Pharyngitis
    1. See Streptococcal Pharyngitis for treatment regimens
  5. Prevention of recurrence
    1. Rheumatic Fever with carditis and residual heart disease (valvcular disease persists on echo or exam)
      1. Benzathine Penicillin G IM monthly for 10 years or until age 40 years (whichever is longer)
    2. Rheumatic Fever with carditis but NO residual heart disease
      1. Benzathine Penicillin G IM monthly for 10 years or until age 21 years (whichever is longer)
    3. Rheumatic Fever without carditis
      1. Benzathine Penicillin G IM monthly for 5 years or until age 21 years (whichever is longer)
    4. References
      1. Gerber (2009) Circulation 119(11): 1541-51 [PubMed]

XI. Prevention

  1. Antibiotics for Streptococcal Pharyngitis
  2. Test and treat household contacts

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Related Studies

Ontology: Rheumatic Fever (C0035436)

Definition (MSH) A febrile disease occurring as a delayed sequela of infections with STREPTOCOCCUS PYOGENES. It is characterized by multiple focal inflammatory lesions of the connective tissue structures, such as the heart, blood vessels, and joints (POLYARTHRITIS) and brain, and by the presence of ASCHOFF BODIES in the myocardium and skin.
Definition (NCI) An inflammatory disorder that follows infection with group A streptococcus. It affects the heart, joints, and subcutaneous tissues. It is manifested with pericarditis, heart murmur, congestive heart failure, polyarthritis, subcutaneous nodules, and erythema marginatum. It is characterized by the formation of granulomatous lesions called Aschoff bodies usually in the heart tissue.
Definition (CSP) febrile disease occurring as a delayed sequela of infections with group A hemolytic streptococci and characterized by multiple focal inflammatory lesions of the connective tissue structures, especially of the heart, blood vessels, and joints (polyarthritis), and by the presence of Aschoff bodies in the myocardium and skin.
Concepts Disease or Syndrome (T047)
MSH D012213
ICD9 390-392.99
ICD10 I00-I02.9 , I00-I02
SnomedCT 274095001, 195528001, 155264006, 155274009, 194718003, 266276003, 26424001, 58718002, 81077008
LNC LA16999-7
English Acute Articular Rheumatism, Acute Articular Rheumatisms, Acute Rheumatic Arthritides, Acute Rheumatic Arthritis, Arthritides, Acute Rheumatic, Arthritis, Acute Rheumatic, Arthritis, Rheumatic, Acute, Articular Rheumatism, Acute, Articular Rheumatisms, Acute, Fevers, Rheumatic, Polyarthritis Rheumatica, Polyarthritis Rheumaticas, Rheumatic Arthritides, Acute, Rheumatic Arthritis, Acute, Rheumatic Fevers, Rheumatism, Acute Articular, Rheumatism, Articular, Acute, Rheumatisms, Acute Articular, Fever, Rheumatic, Acute rheumatic fever NOS, RHEUMATIC ARTHRITIS ACUTE, rheumatic fever, rheumatic fever (diagnosis), Acute rheumatism, Fever, Acute Rheumatic, Rheumatic Fevers, Acute, Acute Rheumatic Fever, Acute Rheumatic Fevers, Fevers, Acute Rheumatic, Rheumatic Fever, Acute, Rheumatic Fever [Disease/Finding], Rheumatic Arthritis, Inflammatory Rheumatism, acute rheumatism, polyarthritis rheumatica, acute rheumatic fever, rheumatism acute, Acute rheumatic fever (I00-I02), Inflammatory rheumatism, Acute rheumatic fever (disorder), Acute rheumatic fever NOS (disorder), Acute rheumatic fever, Rheumatic fever, Active rheumatic fever, Acute articular rheumatism, Acute rheumatic arthritis, Acute rheumatic attack, ARF - Acute rheumatic fever, RhF - Rheumatic fever, Active rheumatic fever (disorder), Acute rheumatic arthritis (disorder), Rheumatic fever (disorder), Rheumatic fever, NOS, Rheumatic Fever, Rheumatic fever, acute, ACUTE RHEUMATIC FEVER, Rheumatic fever, active
Dutch acuut reuma, inflammatoir reuma, reumatische koorts, Acuut reuma, Artritis, acute reumatische, Reuma, acuut
French Rhumatisme aigu, Rhumatisme inflammatoire, Rhumatisme articulaire aigu, Syndrome post-streptococcique, RAA (Rhumatisme Articulaire Aigu)
German akutes rheumatisches Fieber, Entzuendlicher Rheumatismus, rheumatisches Fieber, Arthritis, rheumatische, akute, Gelenkrheumatismus, akuter, Rheumatisches Fieber
Italian Reumatismo acuto, Artrite reumatica acuta, Poliartrite reumatica, Reumatismo articolare acuto, Reumatismo infimmatorio, Febbre reumatica
Portuguese Reumatismo agudo, Febre Reumática Aguda, Reumatismo inflamatório, Febre reumática, Artrite Reumática Aguda, Febre Reumática, Reumatismo Articular Agudo
Spanish Reumatismo agudo, Fiebre Reumática Aguda, Reumatismo inflamatorio, fiebre reumática aguda, SAI (trastorno), fiebre reumática aguda, SAI, Acute rheumatic fever NOS, artritis reumática aguda (trastorno), artritis reumática aguda, ataque reumático agudo, fiebre reumática (trastorno), fiebre reumática activa (trastorno), fiebre reumática activa, fiebre reumática aguda, fiebre reumática, reuma articular agudo, reumatismo articular agudo, Fiebre reumática, Artritis Reumática Aguda, Fiebre Reumática, Reumatismo Articular Agudo
Swedish Reumafaktor
Japanese リウマチネツ, キュウセイリウマチネツ, 関節炎-リウマチ-急性, リウマチ熱, 炎症性リウマチ, エンショウセイリウマチ, 急性リウマチ熱, 急性関節リウマチ, リウマチ-関節-急性, 関節リウマチ-急性
Czech revmatická horečka, Akutní revmatismus, Revmatická horečka, revmatická artritida, revmatická polyartritida, akutní revmatická horečka, zánětlivý revmatismus, Zánětlivý revmatismus
Finnish Reumakuume
Croatian Not Translated[Rheumatic Fever]
Polish Choroba Bouillanda, Choroba reumatyczna, Gorączka reumatyczna
Hungarian Rheumás láz, Akut rheumatismus, Gyulladásos rheumatismus
Norwegian Revmatisk artritt, akutt, Revmatisk feber, Akutt artikulær revmatisme, Reumatisk feber, Revmatoid polyartritt, Reumatisk artritt, akutt, Akutt revmatisk artritt, Akutt revmatisk feber, Reumatoid polyartritt, Akutt artikulær reumatisme, Akutt reumatisk artritt, Akutt reumatisk feber, Giktfeber

Ontology: Rheumatic Heart Disease (C0035439)

Definition (CSP) most important manifestation and sequel to rheumatic fever, consisting chiefly of heart valve deformities.
Definition (MSH) Cardiac manifestation of systemic rheumatological conditions, such as RHEUMATIC FEVER. Rheumatic heart disease can involve any part the heart, most often the HEART VALVES and the ENDOCARDIUM.
Concepts Disease or Syndrome (T047)
MSH D012214
ICD9 398.90
ICD10 I09.9
SnomedCT 266286002, 201793007, 155293006, 194752000, 23685000
English Disease, Rheumatic Heart, Diseases, Rheumatic Heart, Heart Disease, Rheumatic, Heart Diseases, Rheumatic, Rheumatic Heart Diseases, Rheumatic heart disease unspec, Rheumatic heart disease unspecified, Rheumatic heart disease, unspecified, Rheumatic Heart Disease, RHEUMATIC HEART DIS, Rheumatic heart disease NOS, rheumatic heart disease (diagnosis), rheumatic heart disease, Unspecified rheumatic heart disease, RHD, Rheumatic heart dis NOS, Rheumatic Heart Disease [Disease/Finding], carditis rheumatic, rheumatic heart diseases, Disease;rheumatic heart, heart disease rheumatoid, disease fever heart rheumatic, heart disease rheumatic fever, disease heart rheumatic, rhd, fever rheumatic heart disease, rheumatic carditis, rheumatoid heart disease, Rheumatic heart disease NOS (disorder), Rheumatic heart disease unspecified (disorder), Rheumatic carditis (disorder), HEART DISEASE, RHEUMATIC, RHEUMATIC HEART DISEASE, DISEASE, RHEUMATIC HEART, Rheumatic heart disease, Rheumatic pancarditis, Rheumatic carditis, Rheumatic heart disease (disorder), heart; disease, rheumatic, pancarditis; rheumatic, rheumatic; pancarditis, Rheumatic carditis, NOS, Rheumatic heart disease, NOS
Dutch reumatische hartaandoening NAO, RHD, reumatische hartaandoening, niet-gespecificeerd, niet-gespecificeerde reumatische hartaandoening, hart; aandoening, reumatisch, pancarditis; reumatisch, reumatisch; pancarditis, Reumatische hartziekte, niet gespecificeerd (chronisch), reumatische hartaandoening, Hartziekte, reumatische, Reumatische hartziekte
French Maladie de Rougnon-Heberden (MRH), Cardiopathie rhumatismale SAI, Cardiopathie rhumatismale non précisée, Cardiopathie rhumatismale, non précisée, Cardiopathie rhumatismale, Cardite rhumatismale, Rhumatisme cardiaque
German rheumatische Herzerkrankung, unspezifisch, RHD, rheumatische Herzkrankheit NNB, unspezifische rheumatische Herzerkrankung, Rheumatische Herzkrankheit, nicht naeher bezeichnet, rheumatische Herzerkrankung, Rheumatische Herzkrankheit
Italian Cardiopatia reumatica, non specificata, Cardiopatia reumatica non specificata, Cardiopatia reumatica NAS, Cardiopatia reumatica
Portuguese Cardiopatia reumática NE, Cardiopatia reumática, Cardiopatia Reumática
Spanish CR, Cardiopatía reumática NEOM, Cardiopatía reumática no especificada, enfermedad cardíaca reumática, SAI, Rheumatic heart disease NOS, enfermedad cardíaca reumática, SAI (trastorno), enfermedad reumática cardíaca no especificada, enfermedad reumática cardíaca no especificada (trastorno), cardiopatía reumática (trastorno), cardiopatía reumática, carditis reumática, enfermedad cardíaca reumática, Cardiopatía reumática, Cardiopatía Reumática
Japanese リウマチ性心疾患NOS, リウマチ性心疾患、詳細不明, リウマチセイシンシッカンショウサイフメイ, リウマチセイシンシッカンNOS, リウマチセイシンシッカン, リウマチ性心臓疾患, ブイヨー病, リウマチ性心疾患, Bouillaud病, 心臓疾患-リウマチ性
Swedish Reumatisk hjärtsjukdom
Czech revmatická karditida, Revmatická choroba srdeční, blíže neurčená, Blíže neurčená revmatická choroba srdeční, Revmatická choroba srdeční, RCHS, Revmatická choroba srdeční NOS, revmatická srdeční nemoc, revmatická choroba srdeční
Finnish Reumaattinen sydänsairaus
Korean 상세불명의 류마티스 심장병
Polish Zapalenie serca reumatyczne, Choroba reumatyczna serca, Zapalenie mięśnia sercowego gośćcowe
Hungarian Rheumás szívbetegség, Rheumás szívbetegség k.m.n., Nem meghatározott rheumás szívbetegség, Rheumás szívbetgség, nem meghatározott, Rhd
Norwegian Hjertesykdom, reumatisk, Hjertesykdom, revmatisk, Revmatisk hjertesykdom, Reumatisk hjertesykdom

Ontology: Erythema marginatum (C0085659)

Concepts Disease or Syndrome (T047)
ICD10 L53.2
SnomedCT 238817005, 50610002
Italian Eritema marginato, Eritema anulare reumatico
Dutch erythema anulare rheumaticum, erytheem; marginatum, marginatum; erytheem, Erythema marginatum, erythema marginatum
French Erythème annulaire rhumatismal, Erythème marginé
German Erythema annulare rheumaticum, Erythema marginatum
Portuguese Eritema anular reumático, Eritema marginado
Spanish Eritema anular reumático, eritema anular reumático, eritema marginado (trastorno), eritema marginado, Eritema marginado
Japanese 辺縁紅斑, リウマチ性環状紅斑, ヘンエンコウハン, リウマチセイカンジョウコウハン
English erythema marginatum, erythema marginatum (diagnosis), Erythema annulare rheumaticum (disorder), Erythema marginatum, Erythema annulare rheumaticum, Erythema marginatum (disorder), erythema; marginatum, marginatum; erythema
Czech Erythema annulare rheumaticum, Erythema marginatum
Korean 유연성 홍반
Hungarian Erythema annulare rheumaticum, Erythema marginatum