Systemic-onset juvenile idiopathic arthritis (Still's disease)

Question 1

Still’s disease accounts for ___ % of juvenile idiopathic arthritis and is one of the only ones that has skin findings (which is why dermatologists see this)

Answer 1

• 20%

Question 2

_____ is the most common rheumatologic disease in childhood.

Answer 2

Juvenile idiopathic arthritis

Question 3

M:F predominance of Stills disease?

Answer 3

M=F (all other JIA’s have female predominance)

Question 4

By definition Juvenile idiopathic arthritis has an onset at age ____ or less.

Answer 4

16

Question 5

What is pathogenesis of Still’s disease?

Answer 5

autoinflammaotry syndrome (disorder of innate immune system) that leads to increased IL-1 production—> downstream effects., rather than an autoimmune disease of the adaptive immune system

Question 6

Which cytokine is the major driver of inflammation in Still’s disease?

Answer 6

IL-1

Question 7

What are the diagnostic criteria for Still’s disease?

Answer 7

High episodic feveers daily for >/= 2 weeks and documented to be daily for >/= 3 days (classically arises in late afternoon/early evening)

Plus one of the following:

• transient, evanescent, salmon pink, blanching eruption (90%) that correlates with fevers and koebnerizes
• generalized LAD
• Hepatosplenomegaly
• Serositis (pericarditis, pleuritis, perotinitis)
• Symmetric polyarthritis > oligoarthritis (erosive in 20%)

Question 8

Histopath of Still’s disease:

Answer 8

evanescent transient exanthem:

• edema of superficial dermis
• superficial, PV, and interstitial neutrophilic infiltrate in absence of vasculitis

Persistent papules/plaques

• same as above + PK and superficially-scattered necrotic keratinocytes

Question 9

What are the skin findings in stills disease?

Answer 9

• 90% will have transient evanescent, salmon pink, blanching eruption corresponding with fever spikes
• can also see persistent papules and plaques

Question 10

Labs to check in Still disease:

Answer 10

• elevated ESR/CRP

- RF-negative

- ANA negative

Question 11

Are RF and ANA in Stills disease negative or positive?

Answer 11

Both negative 95% of the time

Question 12

Treatment for stills disease if mild? Mod-severe?

Answer 12

For mild: NSAIDS +/- Hydroxychloroquine

For moderate-severe: Systemic steroids +/- steroid sparing immunosuppressants (MTX, TNF-a inhibitors)

• IL-1 Receptor antagonists (Anakinra) and IL-6 receptor antagonists (rocilizumab) show promising results

Question 13

Prognosis of Still’s disease?

Answer 13

Arthritis resolves completely in 50%

• other 50% have chronic course w/ persistent arthtitis and systemic complications

Question 14

Extensive arthritis or symptoms lasting > ______ amount of time is a/w poor prognosis in Stills disease.

Answer 14

6 months