Still's disease (JIA) and Adult Onset Still's Disease

Question 1

Epidemiology: Still’s disease vs. Adult Onset Still’s

Answer 1

the former is < 16 yo, the latter is < 30 yo

Question 2

Adult Onset Still

Answer 2

prodrome of flu-like illness with sore throat, then high fever, and skin finding (either transient, salmon patch exanthema or persistent papules/plaques) +/- hepatosplenomegaly, +/- CARPAL ANKYLOSIS (no pain, just limited ROM, pathognomonic);elevated ESR/CRP and ferritin; negative RF and ANA; Tx: steroids, followed by MTX, anakinra, tocilizumab. concern for macrophage activation syndrome (life threatening) 15% of patients

Question 3

Stills

Answer 3

most common rheum disease in Peds; high episodic fever and skin finding (either transient, salmon patch exanthema or persistent papules/plaques) +/- serositis, hepatosplenomegaly; elevated ESR/CRP and ferritin; negative RF and ANA; Tx: steroids, followed by MTX, anakinra, TNF alpha inhibitors, hematopoietic stem cell transplant. concern for macrophage activation syndrome (life threatening) 5% of patients. pathogenesis: increased IL-1 production