Today, we talked about the very interesting case of a middle-aged man who presented with acute migrating oligoarthritis, found to be febrile with an inflammatory synovial fluid and elevated ASO titers consistent with acute rheumatic fever!

Clinical Pearls

• Nonsuppurative manifestations of GAS infection include acute rheumatic fever (ARF), acute GN, and Scarlet fever.
• Use the modified Jones Criteria to help you diagnose ARF and treat early if high suspicion for the disease (do not wait for titers to come back).
• Late complications of ARF include rheumatic heart disease (10-20 years after infection) and Jaccoud arthropathy.
• Treatment of ARF involves NSAIDs for arthritis, PCN G IM x 1 dose for acute presentation and then monthly for prophylaxis, and patient education about oral hygiene to prevent endocarditis and need for prophylaxis before invasive procedures.

Differential diagnosis for a migratory arthritis

• Rheumatic fever
• Infective endocarditis
• Vasculitis (IgA, cryo, ANCA associated)
• SLE 
• Acute leukemia
• Serum sickness
• Viral arthritis
• Bacteremia (staph, strep, mening/gonococcal)
• Pulmonary infections (mycoplasma, histoplasma)
• Lyme
• Whipple’s

Nonsuppurative complications of GAS infection

• ARF
• Scarlet fever
• Acute GN

Rheumatic fever 

• Nonsuppurative sequela that occurs 2-4 weeks after GAS pharyngitis
• Epi
  • More common in children 5-15 years of age
  • More common in resource limited settings
• Pathogenesis:
  • Poorly understood, ?molecular mimicry
• Clinical manifestations:
  • Two primary manifestations of disease

(Table above from UpToDate)

• Late sequelae
  • Rheumatic heart disease (10-20 years after infection), primary involves the mitral valve >aortic valve.
    • Leading cause of cardiovascular death in the first 5 decades of life in resource limited settings
  • Jaccoud arthropathy
• Diagnosis:
  • Revised Jones criteria (joint and cardiac manifestations can only be counted once).
    • Major
      • Carditis and valvulitis (clinical or subclinical) – 50-70%
        • Usually pancarditis. Valvulitis especially of mitral and aortic valves, shown as regurg on echo.
        • Carey Coombs murmur: short mid-diastolic murmur heard loudest at the apex
      • Arthritis (migratory, involving large joints) – 35-66%, earliest symptom
        • Several joints affected in quick succession, each inflamed for a day or two to one week. Most common are knees, ankles, elbows, and wrists.
      • CNS involvement (Sydenham chorea) – 10-30%
      • Subcutaneous nodules – 0-10%
      • Erythema marginatum – <6% 
    • Minor
      • Arthralgia
      • Fever >38.5
      • Elevated acute phase reactants (ESR, CRP)
      • Prolonged PR interval on EKG
    • Diagnosis requires evidence of prior GAS infection plus:
      • 2 major OR
      • 1 major + 2 minor criteria OR
      • 3 minor criteria (only if patient has history of prior episode of ARF)
    • In a high prevalence setting, slightly modified criteria are used.
  • Labs:
    • Prior GAS infection through either
      • Throat culture
      • Positive rapid strep antigen test
      • Elevated or rising ASO titers
    • Treatment
      • Goals
        • Symptomatic relief of acute disease manifestations
          • Arthritis: NSAIDs
          • Carditis: if severe, heart failure treatments
        • Eradication of GAS
          • IM PCN G benzathine x 1
          • Contacts (throat culture test and treat if positive)
        • Ppx against future GAS infection to prevent progression of cardiac disease
          • PCN G IM once a month
          • For 5 years or until 21 years of age (whichever is longer)
          • If ARF with carditis and residual heart disease
            • 10 years or until 40 years, sometimes even lifelong
          • Education
            • Oral health
            • Ppx before any invasive procedures