Acute Rheumatic Fever 08.13.2012

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    Daniel Tawfik

    8/13/2012

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    10Y 9M year old male in clinic:- 1 month ago developed fever, mouth sores and a rash on his hands; he andhis 2 brothers were diagnosed with hand, foot and mouth disease. Noantibiotics given.

    - 3 weeks ago his legs felt achy with intermittent left hip pain, had fatigue,malaise, and chest pain (worse on walking). Also dizziness when standing for along time.

    - 2 weeks ago he was seen in an OSH ER for abdominal pain; a CT scansuggested mesenteric adenitis.

    - 1 week ago he developed left knee swelling. Arthrocentesis showed negative

    cultures.

    - Nowwith continued lower extremity "achiness" and fatigue. No sore throat,cough, chest pain, or shortness of breath. He has been playing soccer and hasbeen able to go to school.

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    Past Medical History: Full-term birth, no medical problems.He has never been hospitalized. No surgeries.

    Medications: Multivitamin prn, Naproxen and Lortab asneeded for pain.

    Allergies: NKDA

    Immunizations: Up to date per mother.

    Social History: Has a pet fish. Does well in the 5th grade. Nolearning and behavioral problems. Plays soccer and basketball.

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    T 36.0, HR 60, RR 14, BP 124/72 (right arm) and 150/80 (right leg)weight 66.2 kg (99th percentile)height 155 cm (96th percentile)

    General: Well developed, overweight, cooperative, no acute distress.HEENT: nondysmorphic, mucous membranes pink and moist. Inside

    of his eyelids slightly pale. Sclerae anicteric without erythema.Neck: Supple. No JVD in the sitting position. No thyromegaly orcervical lymphadenopathy.Chest: lungs clear to auscultation, good air entry bilaterally.Cardiovascular: Prominent PMI. Normal S1, single S2. Grade III/VIholodiastolic high-pitched murmur, heard best along the left sternalborder. No systolic murmur. Pulses 2 to 3+ throughout.

    Abdomen: Soft, nontender, nondistended. Liver palpable 2 cm belowthe right costal margin. No splenomegaly.Extremities: No cyanosis, clubbing, or edema.Skin: Mild eczema throughout.Neurologic: Grossly intact. Normal gait, tone, and affect.

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    Acute rheumatic feverReactive arthritis

    JIALymphomaTraumaLeukemia

    Lyme diseaseParvovirusSLELegg-Calve-PerthesSCFEEndocarditisOsteomyelitis

    Septic arthritisCostochondritis

    Acute Chest SyndromePericarditis

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    Arthrocentesis: WBC 6550 (79% PMNs), RBC 1CMP: normal

    WBC 5.6, Hgb 10.4, Hct 30.6, Plts 304ESR 96CRP 6

    UA: 1+ ketones, trace Hgb, 2+ protein Rapid strep: negative TTE: Mild tricuspid insufficiency. Dilated left atrium. Mild

    mitral valve insufficiency. Moderate to severe (3-4+) aorticvalve regurgitation, flow reversal in the abdominal aorta.

    Normal biventricular size and qualitatively normal systolicfunction. ASO 2041

    DNAse B 2130

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    Epidemiology Primarily children 5-15 years old

    19 per 100,000 worldwide, 2-14 per 100,000 in developedcountries

    More prevalent in Utah (and Trinidad and Hawaii)

    2-4 weeks after GAS pharyngitis (this is why we treatStrep throat) will have had asymptomatic Streptococcal infection.

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    Major Features

    Migratory arthritis

    Carditis/Valvulitis

    CNS involvement

    Erythema marginatumSubcutaneous nodules

    Minor Features

    Arthralgia (in absence of arthritis)

    Fever

    Elevated ESR/CRP

    Prolonged PR interval (in absence of carditis)

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    Migratory arthritis (large joints) 70% Usually first manifestation, most common in older kids NSAIDs work extremely well, can mask migration of arthritis

    Carditis/valvulitis 50% New murmur

    Mitral/Aortic regurgitation on echo CNS involvement (Syndenham chorea) 15%

    Abrupt involuntary movements, often asymmetric Up to 8 months after streptococcal infection

    Erythema marginatum -

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    Evidence of Strep throat Positive rapid test

    Positive throat culture

    Elevated/rising ASO titer (or anti-DNAse B,streptokinase, antihyaluronidase antibodies)

    Diagnostic criteria 2 majors

    1 major + 2 minors

    Sydenham chorea

    Prior ARF and 1 major or 2 minors

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    Antibiotics same as for acute Strep pharyngitis Oral Penicillin, Oral Amoxicillin, IM Penicillin, or

    Cephalexin

    (Azithromycin or Clindamycin if allergic)

    Throat cultures of all household contacts, and treatmentof all positive results. (specific to ARF contacts)

    High-dose aspirin (80-100 mg/kg/day) until symptomsresolved or inflammatory markers normalized Can also use corticosteroids as anti-inflammatory

    Valve repair or replacement if medical heart failuremanagement unsuccessful

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    Prophylaxis duration Residual valve disease: until 40yo (and at least 10 years)

    Resolved valve disease: until 21yo (and at least 10 years)

    No valve disease: until 21yo (and at least 5 years)

    Antibiotic choice IM Penicillin G every 4 weeks (preferred)

    Oral Penicillin V or Sulfadiazene daily (alternatives)

    Azithromycin daily (if allergic)

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    Treat Streptococcal pharyngitis to prevent acuterheumatic fever However, some cases will be asymptomatic

    Suspect ARF with migratory arthritis or new murmur2-4 weeks after an acute illness.

    Treat ARF with Penicillin and anti-inflammatorymedications

    After ARF, antibiotic prophylaxis for at least 5 years,and up to age 40 in selected patients.

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    Bisno AL et al. Practice Guidelines for the Diagnosisand Management of Group A StreptococcalPharyngitis. CID 2002; 35:113-25.

    Carapetis JR. Acute rheumatic fever. Lancet 2005;366:155-68.

    Park MK. Pediatric Cardiology for Practitioners, 3rd 3d.Mosby, St. Louis: 1996.

    Gerber MA. Prevention of Rheumatic Fever andDiagnosis and Treatment of Acute StreptococcalPharyngitis. Circulation 2009; 119:1541-51.