NextGen 2024: Refractory SJIA & MAS Session Part 7

    NextGen 2024: Refractory SJIA & MAS Session Part 7

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    @SJIA_Foundation
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    6 months ago 331

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    Key Insights

    Refractory disease
(Still’s +/- MAS)
Juan Manuel Mosquera A. 
Pediatric Rheumatologist
Attending physician at Pediatric 
Rheumatology section in Sant Joan de 
Déu Children’s Hospital
    1/4
    • ATS, 11 yr, , 33 kg, latino origin
• Cholinergic urticaria and atopic dermatitis
• Typical Still onset – MAS (Fever, arthralgia, sore throat, rash)
• ↑WBC, ESR, CRP, ferritin, LFT, S100A8/A9, IL18 and triglycerids, ↓ PLT, fibrinogen. HLA-DRB1*15
• NGS panel for AID (Heterozygous DNASE2 c.995C>A VUS and PSMB8 c.-5C>T VUS) and PID performed
• HRCT negative
• Antiviral and antibacterial prophylaxis
CS + ANK + CsA + IVIG
Emapalumab (+CS + ANK + CsA)
CS + ANK + CsA
TCZ + CsA (+CS)
TCZ (+CS) 
TK + IVIG (+TCZ + CS)
SARS-CoV2
CMV reactivation
Adenovirus
SARS-CoV2
Adenovirus
CMV reactivation
Rhino/Enterovirus
Rhino/Enterovirus
Rhino/Enterovirus
    2/4
    • JMA, , 13 yr, 45 kg, Philippine origin. Any relevant past history
• Typical Still disease onset (fever, sore throat, myalgia, rash, serositis, w/o arthritis)
• ↑WBC, ESR, CRP, ferritin, LFT, S100A8/A9, IL18 and 🡪 PLT, fibrinogen, triglycerids. HLA-DRB1*15
• HRCT negative
• DRESS like reaction (26/05/24) 🡪 Rash and ↑LFT
IV MP pulses + oral CS 
Anakinra 2,2 mg/kg (+ CS)
Tocilizumab 8 mg/kg (+ CS)
Tacrolimus 0,1 mg/kg (+ CS)
Baricitinib 4 mg/d (+ TK + CS)
    3/4
    Juanmanuel.mosquera@sjd.es
Enfermedades 
Autoinmunes y
Autoinflamatorias
XUEC en malalties 
autoimmunes 
sistèmiques I
autoinflamatòries
    4/4

    NextGen 2024: Refractory SJIA & MAS Session Part 7

    • 1. Refractory disease (Still’s +/- MAS) Juan Manuel Mosquera A. Pediatric Rheumatologist Attending physician at Pediatric Rheumatology section in Sant Joan de Déu Children’s Hospital
    • 2. • ATS, 11 yr, , 33 kg, latino origin • Cholinergic urticaria and atopic dermatitis • Typical Still onset – MAS (Fever, arthralgia, sore throat, rash) • ↑WBC, ESR, CRP, ferritin, LFT, S100A8/A9, IL18 and triglycerids, ↓ PLT, fibrinogen. HLA-DRB1*15 • NGS panel for AID (Heterozygous DNASE2 c.995C>A VUS and PSMB8 c.-5C>T VUS) and PID performed • HRCT negative • Antiviral and antibacterial prophylaxis CS + ANK + CsA + IVIG Emapalumab (+CS + ANK + CsA) CS + ANK + CsA TCZ + CsA (+CS) TCZ (+CS) TK + IVIG (+TCZ + CS) SARS-CoV2 CMV reactivation Adenovirus SARS-CoV2 Adenovirus CMV reactivation Rhino/Enterovirus Rhino/Enterovirus Rhino/Enterovirus
    • 3. • JMA, , 13 yr, 45 kg, Philippine origin. Any relevant past history • Typical Still disease onset (fever, sore throat, myalgia, rash, serositis, w/o arthritis) • ↑WBC, ESR, CRP, ferritin, LFT, S100A8/A9, IL18 and 🡪 PLT, fibrinogen, triglycerids. HLA-DRB1*15 • HRCT negative • DRESS like reaction (26/05/24) 🡪 Rash and ↑LFT IV MP pulses + oral CS Anakinra 2,2 mg/kg (+ CS) Tocilizumab 8 mg/kg (+ CS) Tacrolimus 0,1 mg/kg (+ CS) Baricitinib 4 mg/d (+ TK + CS)
    • 4. Juanmanuel.mosquera@sjd.es Enfermedades Autoinmunes y Autoinflamatorias XUEC en malalties autoimmunes sistèmiques I autoinflamatòries


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