NextGen 2024: Weaning meds when disease is controlled Session Part 2

@SJIA_Foundation

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2 weeks ago 167

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NextGen 2024: Weaning meds when disease is controlled Session Part 2

@SJIA_Foundation2 weeks ago

SJIA
Weaning Therapy
Fatma Dedeoglu, MD
Boston Children’s Hospital
Harvard Medical School
11…

1/15

Disclosures
UpToDate (Royalties)
SOBI (Honorarium)

2/15

PATTERNS
Recurrent/Episodic with long off medication
Persistent but with medication free period…

3/15

Patient 1
• 18 yo M
• Dx Still’s
• Very high ESR (130), CRP (25 mg/dl)
• High WBC, plt
• IL-18…

4/15

• 13 yo F Patient 2
• Presented at age 4
• Multiple MAS episodes, evolving into autoimmunity (Sjo…

5/15

Patient 3
• 22 yo F- Still Disease /MAS
• Age 19 y fever, rash, cytopenia, pancreatitis, liver
…

6/15

• Age 3 y
• 7 mo: fever, rash
• ESR:70, CRP:15,
high WBC, plt
• Anakinra
• Wean over 18m
(Q…

7/15

Patient 6
• Age 1
• Started on anakinra (1year)
• flare 18 m later; brief anakinra -> canakinum…

8/15

GAPS
❖ Need to improve interpretation of genetic test
results
❖ Need to understand influence of…

9/15

Example
• This sequence change replaces serine with arginine at codon 1419 of the
FAT4 protein (…

10/15

❖ Need to improve delivery of care
❖ Need to improve preventive care, vaccinations
❖ Need to unde…

11/15

Follow clues
Clinical (similarities to other diseases,
patient you see,
medication respons…

12/15

13
MANAGEMENT
MULTIDISCIPLINARY CARE
RAPID DISEASE CONTROL
PREVENTION OF DAMAGE FROM DISEASE AN…

13/15

14/15

Boston Children’s Hospital (BCH) Rheumatology
Program and Autoinflammatory Clinic staff
THANK YO…

15/15

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NextGen 2024: Weaning meds when disease is controlled Session Part 2

@SJIA_Foundation

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NextGen 2024: Weaning meds when disease is controlled Session Part 2