IL-18 testing in the diagnosis and management of Still’s disease: Game-changer or Red Herring?

IL-18 testing in the diagnosis and management of Still’s disease: Game-changer or Red Herring?

@SJIA_Foundation
@SJIA_Foundation
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2 weeks ago 81

IL-18 testing in the diagnosis and management of Still’s disease: Game-changer or Red Herring?

@SJIA_Foundation2 weeks ago

IL-18 testing in the diagnosis and 
management of Still’s disease:
Game-changer or Red Herring?
…
1/13
Disclosures
• Industry funded research
• Site PI (completed): Novartis, AB2Bio
• In-kind support…
2/13
IL-18 
testing
3/13
A common clinical conundrum
6 mos prev healthy male
- Born FT, no preg/perinatal issues
- Presen…
4/13
5/13
Which is the Still’s 
Rash?
6/13
Still’ s
FMF
TRA
PS HID S
Kawasa
ki
CAP S
???
Stre p
7/13
Presentation 
continued EBV/CMV/Adeno/HHV
6
- PCR neg
Perforin/GranyzmeB: elevated by % & 
MCF…
8/13
FUO with 
HLH
• Prolonged viral 
syndrome
• Still’s
• Atypical KD
• Autoinflammatory
To
‘Ro…
9/13
FUO with 
HLH
• Prolonged viral 
syndrome
• Atypical KD
• Autoinflammatory
• Still’s
• Monog…
10/13
OK, so it’s 
IL-18 = 128,000 Still’s
pg/mL
CXCL9 = 14,000 
pg/mL
sIL-2Ra = 2300 pg/mL
Anakinr…
11/13
Flare upon GC 
wean
Recheck IL-18 to 
determine:
Fever, ferritin, LFTs don’t tolerate < 0.5 mg/…
12/13
Remission on 
medication
Still’s MRD 
biomarkers
• LDH/aldolase
• D-dimer
• Ferritin
• sIL-2…
13/13


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IL-18 testing in the diagnosis and management of Still’s disease:
Game-changer or Red Herring?

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