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    NextGen 2024: Monitoring &Treating Lung Disease in SJIA Session Part 2

    NextGen 2024: Monitoring &Treating Lung Disease in SJIA Session Part 2

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    @SJIA_Foundation
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    5 months ago 372

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    Key Insights

    #Sjiafoundation#Sjia#Curesjia#Lungdisease
    Recognizing and Treating Lung Disease in 
Systemic Juvenile Idiopathic Arthritis
Lauren A. Henderson, MD, MMSc
NextGen Therapies in sJIA, Still’s & MAS
November 14, 2024
    1/11
    Disclosures
• Employment: CARRA, Translational Research Chair
• Industry funded research/investigator: BMS, Adaptive Biotechnologies
• Consultant: Sobi, Pfizer
• Stock/Ownership: None
• Patent holder: None
• Other (please describe): None
• Plan to discuss investigational/off-label uses of drugs or devices?: Yes
    2/11
    Importance of Pulmonary Screening in sJIA
• Lung disease is an increasingly recognized complication of sJIA
• Reported prevalence of sJIA lung disease has ranged from 1.5-20%
• Initially, many patients are asymptomatic
• Affected children are young and have difficulty reporting respiratory symptoms
Wobma H...Henderson LA. Arthritis Care Res 2023
Erkens RGA et al. Arthritis Rheumatol 2024
Zhan W et al. Pediatric Rheumatol 2024 From Schulert GS et al. Arthritis Rheumatol 2019
    3/11
    Importance of Pulmonary Screening in sJIA
A systematic approach to pulmonary screening in sJIA is needed
• To understand the true spectrum and burden of disease
• To identify patients early and initiate treatment to prevent irreversible damage
Case 2
Chest CT 
@ sJIA-LD diagnosis
Stable chest CT 
9mo later
🡪 Tacro added 🡪
Pontes Aires B...Henderson LA. J Rheumatol 2024
Case 1
Chest CT 
@ sJIA-LD diagnosis
Rapid progression 
despite max therapy
🡪
Barbara Pontes Aires
    4/11
    Developing a Pulmonary Screening Guideline for sJIA
Multidisciplinary Workgroup Consensus Process
Wobma H...Henderson LA. ACR Open Rheumatol 2023
Holly Wobma
    5/11
    Developing a Pulmonary Screening Guideline for
sJIA
New onset sJIA 
1
st-line treatment with 
IL-1/IL-6 inhibitor
IL-18 & CXCL9 tracked serially
Send HLA typing
Red Flag Features
1. Age <24 mo @ presentation
2. HLA DRB1*15:XX positive
3. Trisomy 21
4. History of CHD*
5. High disease activity (ICU, MAS, labs)
6. New atypical rash 
7. Eosinophilia
8. Anaphylaxis (any drug)
9. Respiratory symptoms
10. Abnormal pulm exam or clubbing
Assess sJIA-LD Risk
Referral to Pulm Clinic
CXR & PFTs
Schulert GS et al. Arthritis Rheumatol 2019
Kimura Y et al. Arthritis Car Res 2013
Saper VE et al. ARD 2019
Saper VE et al. ARD 2022
Wobma H...Henderson LA. Arthritis Care Res 2023
    6/11
    Developing a Pulmonary Screening Guideline for
sJIA
Referral to Pulm Clinic
CXR
PFTs
(spirometry, lung volumes, 
DLCO 3/6min walk test)
High resolution chest CT & BAL
Modify treatment
F/U in rheum and pulm clinics 
Q2-3 months
F/U in rheum and pulm clinics 
at least Q6 months
Abnormal pulm eval 
AND/OR uncontrolled disease
Normal pulm eval
Progression
    7/11
    Approach to Managing sJIA-LD
Establish Full Remission
• Resolution of clinical symptoms
• Fevers
• Rashes (including pruritic rash)
• Arthritis
• HSM
• Serositis
• MAS
• Eosinophilia
• Normalization of sJIA/MAS biomarkers
• IL-18
• CXCL9
• Ferritin
• sIL2R
• CRP
• LDH
• LFTs, coags, cell counts
Layer Treatments to Gain Disease Control
Anti-IFNγ
JAKi
DMARDs 
(MTX, CsA, Tacro)
bDMARDs & Glucocorticoids
(IL-1/IL-6 inhibitors)
    8/11
    Approach to Managing sJIA-LD
Close Monitoring for ILD Progression
• F/U with pulmonologist who 
understands sJIA-LD
• Serial PFTs (with DLCO and walk tests)
• Serial chest CTs
• Echos to monitor for pulm HTN
Alicia Casey
    9/11
    Approach to Managing sJIA-LD
• Establish relationship with HSCT team willing to manage sJIA-LD
• Consider early referral for education, HLA typing, donor search
• Expedited HSCT referral:
• Patients with many risk factors
• Patients with uncontrolled disease despite maximal therapy
• ILD progression despite maximal therapy
• Patients with comorbidities that may limit transplant options
Referral to HSCT 
Holly Wobma
Susan Prockop
    10/11
    Thank You!
BCH Rheumatology
Holly Wobma
Barbara Pontes Aires
Joyce Chang
Maggie Chang
Mia Chandler
Megan Day-Lewis
Fatma Dedeoglu
Olha Halyabar
Claudia Harris
Daniel Ibanez
Liyoung Kim
Pui Lee
Mindy Lo
Kyle McBrearty
Esra Meidan
Mary Beth Son
Peter Nigrovic
BCH Pulmonary
Alicia Casey
Martha Fishman
Timothy Klouda
Katie Krone
Aida Samad
Jonathan Levin
BCH HSCT
Susan Prockop
BCH Cardiology
Susan Saleeb
Wayne Tworetzky
Jane Newburger
The patients and families that contributed to and inspired this work
    11/11

    NextGen 2024: Monitoring &Treating Lung Disease in SJIA Session Part 2

    • 1. Recognizing and Treating Lung Disease in Systemic Juvenile Idiopathic Arthritis Lauren A. Henderson, MD, MMSc NextGen Therapies in sJIA, Still’s & MAS November 14, 2024
    • 2. Disclosures • Employment: CARRA, Translational Research Chair • Industry funded research/investigator: BMS, Adaptive Biotechnologies • Consultant: Sobi, Pfizer • Stock/Ownership: None • Patent holder: None • Other (please describe): None • Plan to discuss investigational/off-label uses of drugs or devices?: Yes
    • 3. Importance of Pulmonary Screening in sJIA • Lung disease is an increasingly recognized complication of sJIA • Reported prevalence of sJIA lung disease has ranged from 1.5-20% • Initially, many patients are asymptomatic • Affected children are young and have difficulty reporting respiratory symptoms Wobma H...Henderson LA. Arthritis Care Res 2023 Erkens RGA et al. Arthritis Rheumatol 2024 Zhan W et al. Pediatric Rheumatol 2024 From Schulert GS et al. Arthritis Rheumatol 2019
    • 4. Importance of Pulmonary Screening in sJIA A systematic approach to pulmonary screening in sJIA is needed • To understand the true spectrum and burden of disease • To identify patients early and initiate treatment to prevent irreversible damage Case 2 Chest CT @ sJIA-LD diagnosis Stable chest CT 9mo later 🡪 Tacro added 🡪 Pontes Aires B...Henderson LA. J Rheumatol 2024 Case 1 Chest CT @ sJIA-LD diagnosis Rapid progression despite max therapy 🡪 Barbara Pontes Aires
    • 5. Developing a Pulmonary Screening Guideline for sJIA Multidisciplinary Workgroup Consensus Process Wobma H...Henderson LA. ACR Open Rheumatol 2023 Holly Wobma
    • 6. Developing a Pulmonary Screening Guideline for sJIA New onset sJIA 1 st-line treatment with IL-1/IL-6 inhibitor IL-18 & CXCL9 tracked serially Send HLA typing Red Flag Features 1. Age <24 mo @ presentation 2. HLA DRB1*15:XX positive 3. Trisomy 21 4. History of CHD* 5. High disease activity (ICU, MAS, labs) 6. New atypical rash 7. Eosinophilia 8. Anaphylaxis (any drug) 9. Respiratory symptoms 10. Abnormal pulm exam or clubbing Assess sJIA-LD Risk Referral to Pulm Clinic CXR & PFTs Schulert GS et al. Arthritis Rheumatol 2019 Kimura Y et al. Arthritis Car Res 2013 Saper VE et al. ARD 2019 Saper VE et al. ARD 2022 Wobma H...Henderson LA. Arthritis Care Res 2023
    • 7. Developing a Pulmonary Screening Guideline for sJIA Referral to Pulm Clinic CXR PFTs (spirometry, lung volumes, DLCO 3/6min walk test) High resolution chest CT & BAL Modify treatment F/U in rheum and pulm clinics Q2-3 months F/U in rheum and pulm clinics at least Q6 months Abnormal pulm eval AND/OR uncontrolled disease Normal pulm eval Progression
    • 8. Approach to Managing sJIA-LD Establish Full Remission • Resolution of clinical symptoms • Fevers • Rashes (including pruritic rash) • Arthritis • HSM • Serositis • MAS • Eosinophilia • Normalization of sJIA/MAS biomarkers • IL-18 • CXCL9 • Ferritin • sIL2R • CRP • LDH • LFTs, coags, cell counts Layer Treatments to Gain Disease Control Anti-IFNγ JAKi DMARDs (MTX, CsA, Tacro) bDMARDs & Glucocorticoids (IL-1/IL-6 inhibitors)
    • 9. Approach to Managing sJIA-LD Close Monitoring for ILD Progression • F/U with pulmonologist who understands sJIA-LD • Serial PFTs (with DLCO and walk tests) • Serial chest CTs • Echos to monitor for pulm HTN Alicia Casey
    • 10. Approach to Managing sJIA-LD • Establish relationship with HSCT team willing to manage sJIA-LD • Consider early referral for education, HLA typing, donor search • Expedited HSCT referral: • Patients with many risk factors • Patients with uncontrolled disease despite maximal therapy • ILD progression despite maximal therapy • Patients with comorbidities that may limit transplant options Referral to HSCT Holly Wobma Susan Prockop
    • 11. Thank You! BCH Rheumatology Holly Wobma Barbara Pontes Aires Joyce Chang Maggie Chang Mia Chandler Megan Day-Lewis Fatma Dedeoglu Olha Halyabar Claudia Harris Daniel Ibanez Liyoung Kim Pui Lee Mindy Lo Kyle McBrearty Esra Meidan Mary Beth Son Peter Nigrovic BCH Pulmonary Alicia Casey Martha Fishman Timothy Klouda Katie Krone Aida Samad Jonathan Levin BCH HSCT Susan Prockop BCH Cardiology Susan Saleeb Wayne Tworetzky Jane Newburger The patients and families that contributed to and inspired this work


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