Updated: 10/2/2018

Rheumatic Fever

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Snapshot
  • A 10-year-old girl presents with a fever and migrating joint pains in the knees and elbows. She reports having had a sore throat a few days ago. On physical exam, she is febrile and has barely raised erythematous serpentine-like lesions on her back. A rapid streptococcal test is positive and there is an elevated erythrocyte sedimentation rate. She is given antibiotics for this disease.
Introduction
  • Clinical definition
    • acute inflammation of multiple systems caused by an immunologic reaction to group A streptococcus infection
  • Epidemiology
    • demographics
      • children and adolescents
      • most common in developing nations
    • location
      • mitral valve > aortic valve > tricuspid valve
      • most commonly affects the high-pressure valves
    • risk factors
      • poverty and overcrowding
      • preceding group A streptococcal pharyngitis
  • Etiology
    • typically due to inadequate treatment of group A streptococcal infection
  • Pathogenesis
    • abnormal immune response to group A streptococci
      • molecular mimicry between streptococcal M protein and self-proteins including cardiac proteins, keratin, laminin, and vimentin
      • immune-mediated (type II) hypersensitivity
    • disease is characterized by
      • carditis
      • arthritis
      • Sydenham chorea
        • caused by antibodies that cross the blood-brain barrier and bind dopamine D1 and D2 receptors
      • erythema marginatum
      • subcutaneous nodules
  • Associated conditions
    • Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infection (PANDAS)
      • believed to be an autoimmune condition caused by antibodies targeting the basal ganglia after group A streptococcal infection
      • resulting in acute onset of obsessive compulsive disorder and/or tics
  • Prognosis
    • occurs 2-3 weeks after pharyngitis, except carditis and chorea
      • carditis occurs after months
      • chorea presents after months
    • risk of recurrence decreases with time
Presentation
  • Symptoms 
    • migratory joint pains, especially in the knees, ankles, and elbows
    • chest discomfort
    • heart failures (rare)
  • Physical exam
    • fever
    • may have dyspnea
    • new murmur on cardiac exam may indicate carditis
    • firm and painless subcutaneous nodules over bony prominences
      • often occurs with carditis
    • erythema marginatum
      • evanescent flat or barely raised ring-like or serpentine lesion on trunk
    • Sydenham chorea
      • involuntary, jerky, purposeless movements of the hands, feet, face, or tongue
Imaging
  • Echocardiography
    • indications
      • when murmur is auscultated on examination suspicious for rheumatic heart disease
      • to confirm complication of acute rheumatic fever
    • findings
      • valvular abnormalities, including regurgitations or stenosis
Studies
  • Positive throat culture or rapid antigen test for Streptococcus
  • Labs
    • ↑ anti-streptolysin O (ASO) titers
    • ↑ anti-deoxyribonuclease B titers
  • Electrocardiography
    • prolonged PR interval
      • first-degree heart block
  • Histology
    • Aschoff bodies
      • granuloma with giant cells on heart valves
    • Anitschkow cells
      • enlarged macrophages within Aschoff bodies
  • Making the diagnosis
    • based on clinical presentation, clinical or laboratory evidence of preceding group A streptococcal infection, and confirmation with 2 major Jones criteria or 1 major and 1 minor
 
Jones Criteria
Major Criteria
Minor Criteria
  • Joints (migratory arthritis)
  • O looks like the heart (carditis)
  • Nodules (subcutaneous)
  • Erythema marginatum
  • Sydenham chorea
  • Fever
  • Antecedent strep infection
  • Arthralgias
  • ↑ Erythrocyte sedimentation rate
  • ↑ C-reactive protein
  • First-degree heart block
 
Differential
  • Infective endocarditis
    • distinguishing factors
      • no association with group A streptococcal infection
      • other findings including Roth spots, Osler nodes, Janeway lesions, and splinter hemorrhages on nail bed
      • vegetations seen on valves on imaging
Treatment
  • Management approach
    • patients must be treated adequately with antibiotics to prevent rheumatic heart disease
    • patients must then be put on long-term prophylaxis to prevent recurrence
  • Medical
    • penicillins
      • indication
        • for all patients
    • macrolides
      • indications
        • for patients allergic to penicillin
    • nonsteroidal anti-inflammatory drugs (NSAIDs)
      • indication
        • for patients with joint pain or fever
Complications
  • Rheumatic heart disease
 

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Questions (1)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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