Adult onset Still's disease

Question 1

Age group for Adult onset Still’s disease?

Answer 1

• Vast majority are < 30

Question 2

Pathogenesis of adult onset Still’s disease

Answer 2

possibly a reactive condition triggered by infectious agent

Question 3

Clinical presentation of adult onset Still’s disease:

Answer 3

• prodrome of flu-like illness w/ sore throat, constitutional symptoms, high fever, arthralgias and myalgias
• fever usually spikes in the late afternoon/early evening (same as in juvenile form)

Question 4

Describe the fever of adult onset Still’s disease

Answer 4

• high grade and spikes in afternoon/early evening

Question 5

What are the skin manifestations of adult onset Still’s disease?

Answer 5

• Salmon patch exanthema (asymptomatic and transient)
• occurs along with fever spikes and can koebnerize
• (same as juvenile form)
• can also have red-brown scaly persistent papules and plaques

Question 7

What are the systemic manifestations of adult onset Still’s disease?

Answer 7

• arthralgias
• Carpal ankylosis (characteristic feature): limited range of motion with minimal pain
• Hepatosplenomegaly

Question 8

What is a rare but life threatening complication of adult onset Still’s disease?

Answer 8

Macrophage activation syndrome

Question 9

Histopath of adult onset Still’s disease:

Answer 9

• same as Juvinile/classic stills

Question 10

Lab findings of adult onset Still’s disease

Answer 10

• negative ANA and RF
• anemia, leukocytosis and thrombocytosis common
• very high ferritin!! that correlates w/ disease activity

Question 11

What lab marker correlates with disease activity in adult onset Still’s disease?

Answer 11

Ferritin (can be extremely high)

Question 12

Tx of adult onset Still’s disease

Answer 12

majority require systemic steroids +/- MTX

• may get some benefit with IL-1 or IL-6 inhibitors (anakinra/tocilizumab)

Question 13

prognosis of adult onset Still’s disease

Answer 13

• Usually benign, non-fatal course with low mortality