Journal article
Disease course, treatments, and outcomes of children with systemic juvenile idiopathic arthritis associated lung disease ( SJIA‐LD )
Arthritis care & research (2010), Vol.76(3), pp.328-339
03/2024
DOI: 10.1002/acr.25234
PMCID: PMC11156420
PMID: 37691306
Abstract
Objective Systemic juvenile idiopathic arthritis‐associated lung disease (SJIA‐LD) is a life‐threatening disease complication. Key questions remain regarding clinical course and optimal treatment approaches. The objectives of the study were to detail management strategies after SJIA‐LD detection, characterize overall disease courses, and measure long‐term outcomes. Methods This was a prospective cohort study. Clinical data was abstracted from the electronic medical record including current clinical status and changes since diagnosis. Serum biomarkers were determined and correlated with presence of LD. Results We enrolled 41 SJIA‐LD patients, 85% with at least one episode of macrophage activation syndrome, and 41% with adverse reactions to a biologic. Although 93% of patients were alive at last follow‐up (median 2.9 years), 37% progressed to requiring chronic oxygen or other ventilator support, and 65% of patients had abnormal overnight oximetry studies, which changed over time. 84% of patients carried the HLA‐DRB1*15 haplotype, significantly more than patients without LD. Patients with SJIA‐LD also showed markedly elevated serum IL‐18, variable CXCL9, and significantly elevated MMP‐7. Treatment strategies showed variable use of anti‐IL‐1/6 biologics and addition of other immunomodulatory treatments and lung‐directed therapies. We found a broad range of current clinical status independent of time from diagnosis or continued biologic treatment. Multidomain measures of change showed imaging features were the least likely to improve with time. Conclusion Patients with SJIA‐LD had highly varied courses, with lower mortality than previously reported but frequent hypoxia and requirement for respiratory support. Treatment strategies were highly varied, highlighting an urgent need for focused clinical trials.
Details
- Title: Subtitle
- Disease course, treatments, and outcomes of children with systemic juvenile idiopathic arthritis associated lung disease ( SJIA‐LD )
- Creators
- Yannan Huang - Cincinnati Children's Hospital Medical CenterLaura Sompii-Montgomery - Cincinnati Children's Hospital Medical CenterJessie Patti - Cincinnati Children's Hospital Medical CenterAlex Pickering - Harvard UniversityShima Yasin - University of IowaThuy Do - Cincinnati Children's Hospital Medical CenterElizabeth Baker - Cincinnati Children's Hospital Medical CenterDenny Gao - Cincinnati Children's Hospital Medical CenterRaheh Abdul-Aziz - Department of Pediatric Rheumatology University at Buffalo, Oishei Children's Hospital BuffaloEdward M Behrens - Children's Hospital of PhiladelphiaScott Canna - Children's Hospital of PhiladelphiaMatthew Clark - Prisma HealthDominic O Co - University of Wisconsin–MadisonKathleen P Collins - University of Tennessee Health Science CenterBarbara Eberhard - Cohen Children's Northwell Health New Hyde Park NYMonica Friedman - Arnold Palmer Hospital for ChildrenThomas B. Graham - Monroe Carell Jr. Children's HospitalTimothy Hahn - Penn State Milton S. Hershey Medical CenterAimee O Hersh - University of UtahPatricia Hobday - University of MinnesotaMichael J. Holland - Children's Mercy HospitalJennifer Huggins - Cincinnati Children's Hospital Medical CenterPai‐Yue Lu - University of KentuckyMelissa L Mannion - University of Alabama at BirminghamCynthia K Manos - Children's Healthcare of AtlantaJessica Neely - University of California, San FranciscoKaren Onel - Hospital for Special SurgeryAmir B Orandi - Mayo ClinicAndrea Ramirez - Baylor College of MedicineAdam Reinhardt - Boys Town National Research HospitalMona Riskalla - University of MinnesotaLaisa Santiago - Johns Hopkins All Children's HospitalMatthew L Stoll - University of Alabama at BirminghamTracy Ting - Cincinnati Children's Hospital Medical CenterAlexei A Grom - University of Cincinnati Medical CenterChris Towe - University of Cincinnati Medical CenterGrant S Schulert - Cincinnati Children's Hospital Medical Center
- Resource Type
- Journal article
- Publication Details
- Arthritis care & research (2010), Vol.76(3), pp.328-339
- DOI
- 10.1002/acr.25234
- PMID
- 37691306
- PMCID
- PMC11156420
- NLM abbreviation
- Arthritis Care Res (Hoboken)
- ISSN
- 2151-464X
- eISSN
- 2151-4658
- Grant note
- DOI: 10.13039/100000069, name: National Institute of Arthritis and Musculoskeletal and Skin Diseases, award: K08‐AR072075
- Language
- English
- Electronic publication date
- 09/10/2023
- Date published
- 03/2024
- Academic Unit
- Stead Family Department of Pediatrics
- Record Identifier
- 9984463079502771
Metrics
6 Record Views