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

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
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
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 1

🡪

Chest CT @ sJIA-LD diagnosis

Rapid progression despite max therapy



Case 2

🡪 Tacro added 🡪

Chest CT @ sJIA-LD diagnosis



Developing a Pulmonary Screening Guideline for sJIA

Multidisciplinary Workgroup

Consensus Process



Wobma H...Henderson LA. ACR Open Rheumatol 2023


Developing a Pulmonary Screening Guideline for sJIA


Developing a Pulmonary Screening Guideline for sJIA


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


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

Approach to Managing sJIA-LD

Referral to HSCT

• · 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

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

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

Peter Nigrovic