AM Report 1/18/17: Adult Onset Still’s Disease

Fever of Unknown Origin Criteria:

  • Fever > 38.3 on several occasions
  • Duration of fever > 3 weeks
  • Uncertain diagnosis after 1 week of inpatient investigation

5 Main Etiologies for FUO:

  • Infection
  • Malignancy
  • Connective tissue disease
  • Other
  • No diagnosis

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Adult Onset Still’s Disease (AOSD):

  • Very uncommon – estimated annual incidence of 0.16 cases per 100,000 people
  • Equal distribution between the sexes
  • Bimodal age distribution: one peak between 15-25 and the other between 36-46 years old
  • Diagnosis of exclusion, must exclude infection, malignancy, or other rheumatologic disease.

AOSD is multisystem inflammatory disease characterized by 4 major findings:

  1. High spiking fevers
  2. Salmon-color rash
  3. Arthritis
  4. High neutrophil counts

AOSD Laboratory Findings:

  • Elevated ESR / CRP (99%)
  • Elevated Ferritin (~75%)
  • Leukocytosis > 10,000/mm3 (92%), >15,000/mm3 (81%) with neutrophil predominance (>80%)
  • Abnormal LFTs (~ 75%)
  • Negative ANA (92%)
  • Negative RF (93%)

Yamaguchi Criteria for AOSD:

  • Diagnosis requires 5 criteria total – with at least 2 major criteria

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AOSD Treatment:

Initial therapeutic decisions should be based on the degree of disease activity:

  • Mild disease: fevers, rash, and/or mild arthritis
  • Moderate disease: high fevers, debilitating joint symptoms, evidence of internal organ involvement that is not life-threating or severe
  • Severe disease: presence of life-threating organ involvement

Treatment options:

  • NSAIDS – effective alone for 20% of mild patients
  • Glucocorticoids – should be started in all patients meeting moderate disease criteria
  • Methotrexate – used in patient’s who fail to improve with steroids
  • Can also consider other DMARDs – will not be discussed here!

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