Adult Onset Still’s Disease

Thanks to Dr. Szumowski for sending us the case of a middle aged man who presented with acute L knee swelling and pain one week after a viral URI syndrome, initially concerning for septic joint.  His clinical course was complicated by recurrent high daily fevers, a diffuse maculopapular rash, and knee arthrocenteses and joint washes that were clean leading to a diagnosis of Still’s disease!


Clinical Pearls

  • Still’s disease is a diagnosis of exclusion! Yamaguchi criteria can help with ruling in the diagnosis.
  • Still’s remains a multi-systemic disorder of unknown etiology because it’s difficult to diagnose and rare (0.16 cases per 100,000).
  • RF and ANA are generally negative but can be positive in <10% of patients with Still’s in low titers.
  • ~66% of patients present with sore throat secondary to cricothyroid perichondritis or aseptic nonexudative pharyngitis.
  • The disease is often recurrent. Predictors of poor outcome include erosive polyarthritis on presentation and shoulder/hip involvement.

Still’s disease:

  • Described in 1897 by George Still, it is a systemic inflammatory disorder of unknown etiology
  • Some clarifications on nomenclature:
    • Systemic juvenile idiopathic arthritis (sJIA): first presentation <17 years old, previously referred to as Still’s disease
    • Adult onset Still’s disease (AOSD): first presentation  > 17 years old
  • Epidemiology of AOSD:
    • 0.16 cases per 100,000
    • No sex predominance (F=M)
    • Bimodal age distribution with peak between 15-25 and another 36-46 years of age.  New diagnosis in patients >60 have been reported.
  • Etiology:
    • Poorly understood but likely a combination of genetic predisposition, environmental triggers (viruses such as echo, coxsackievirus B4, mycoplasma, yernisnia, lyme, etc), activated innate immunity leading aberrant production of pro-inflammatory cytokines
  • Diagnostics:
    • High ESR and CRP
    • Very high ferritin levels
    • Ultimately a clinical diagnosis so it’s important to exclude potential mimickers
      • Yamaguchi criteria are the most sensitivity (93.5%)
      • Fautrel’s Criteria are the most specific (98.5%)Diagnostic criteria
  • Treatment
    • Largely empirical since clinical trial data is lacking
    • High dose steroids are first line when systemic symptoms predominate
    • MTX is second line
    • NSAIDs are not good
    • Biologic agents for refractory cases (IL1 antagonist anakinra or canakinumab), IL6 antagonist tocilizumab, or TNF inhibitors.
  • Course:
    • Monocyclic pattern (systemic single episode)
    • Polycyclic pattern (multiple episodes, usually <1 /year)
    • Chronic pattern (persistently active disease with poly arthritis)

      AOSD patterns
      Giacomelli, R. et al. Journal of Autoimmunology. 2018
  • Prognosis:
    • Poor prognostic indicators:
      • Hip and shoulder involvement
      • Erosive polyarthritis at initial diagnosis

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