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

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