Still's disease

Question 1

What is Still disease?

Answer 1

Systemic onset juvenile idiopathic arthritis

Question 2

What are the key features of Still Disease?

Answer 2

Fever
Lymphadenopathy
Hepatosplenomegaly
Arthritis
Evanescent, erythematous eruption that typically accompanies spiking fevers

Question 3

Mean age of onset of Still disease? Sex predilection?

Answer 3

6 years

M = F

Question 4

Clinical presentation of Still Disease?

Answer 4

Daily high fevers (usually above 38.9) - typically later afternoon / evening

Polyarticular arthritis (wrists, knees, ankles, hips, small joints of the hands)

Question 5

Is the arthritis is still
s disease erosive?

Answer 5

20% have erosive disease

Question 6

What are the cutaneous features of still disease?

Answer 6

Exanthem - present in 90% of patients

Most commonly:
Transient
Non-pruritic
Erythematous
Coincides with fevers

Predilication for the axillae and the waist
Can have koebnerisation

Rarely
- more persistent plaques
- periorbital erythematous oedema
- rheumatoid nodule like lesions

Question 7

Describe

Answer 7

Evanescent pink papules and figurate plaques

Question 8

Pathology of stills disease?

Answer 8

perivascular and interstitial neutrophil dominant mixed infiltrate

Variable number of dyskeratotic keratinocytes

Question 9

DDx of Stills disease?

Answer 9

Rheumatic fever
Hypocomplementemic urticarial vasculitis
Serum sickness like reaction
Hereditary periodic fever syndromes

Question 10

Treatment of Stills

Answer 10

IL-1 inhibitors (Anakinra) - is typically first line

NSAIDs
Systemic corticosteroids

Question 11

Who does adult onset still disease affect?

Answer 11

Young adults (before 30)

Slight female predominance

Question 12

A 25 year old women presents with high fevers (in the late afternoon), transient erythematous papules, and arthritis

What is your prefered Dx?

Answer 12

Adult onset stills