Disease Course, Treatments, and Outcomes of Children With Systemic Juvenile Idiopathic Arthritis–Associated Lung Disease

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, ch...

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Published in:Arthritis care & research (2010) Vol. 76; no. 3; pp. 328 - 339
Main Authors: Huang, Yannan, Sompii‐Montgomery, Laura, Patti, Jessica, Pickering, Alex, Yasin, Shima, Do, Thuy, Baker, Elizabeth, Gao, Denny, Abdul‐Aziz, Rabheh, Behrens, Edward M., Canna, Scott, Clark, Matthew, Co, Dominic O., Collins, Kathleen P., Eberhard, Barbara, Friedman, Monica, Graham, Thomas B., Hahn, Timothy, Hersh, Aimee O., Hobday, Patricia, Holland, Michael J., Huggins, Jennifer, Lu, Pai‐Yue, Mannion, Melissa L., Manos, Cynthia K., Neely, Jessica, Onel, Karen, Orandi, Amir B., Ramirez, Andrea, Reinhardt, Adam, Riskalla, Mona, Santiago, Laisa, Stoll, Matthew L., Ting, Tracy, Grom, Alexei A., Towe, Christopher, Schulert, Grant S.
Format: Journal Article
Language:English
Published: Boston, USA Wiley Periodicals, Inc 01.03.2024
Wiley Subscription Services, Inc
Subjects:
Arthritis
Arthritis, Juvenile - complications
Arthritis, Juvenile - diagnosis
Arthritis, Juvenile - drug therapy
Cell activation
Chemokines
Child
Clinical trials
Diagnosis
Disease Progression
Drb1 protein
Electronic medical records
Haplotypes
Humans
Hypoxia
Immunomodulation
Lung
Lung Diseases
Macrophage Activation Syndrome - diagnosis
Macrophage Activation Syndrome - etiology
Macrophage Activation Syndrome - therapy
Macrophages
Matrilysin
Matrix metalloproteinase
Metalloproteinase
Patients
Prospective Studies
ISSN:2151-464X, 2151-4658, 2151-4658
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Summary: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 were ed 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 patients with SJIA‐LD, 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. Eighty‐four percent of patients carried the HLA‐DRB1*15 haplotype, significantly more than patients without LD. Patients with SJIA‐LD also showed markedly elevated serum interleukin‐18 (IL‐18), variable C‐X‐C motif chemokine ligand 9 (CXCL9), and significantly elevated matrix metalloproteinase 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.
Bibliography:Supported by an ARC Award by the Cincinnati Children's Research Foundation, the Systemic Juvenile Idiopathic Arthritis Foundation, and the Jellen Family Foundation. Dr. Schulert was supported by National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health (grant K08‐AR‐072075). Processing and storage of biosamples was supported by the Cincinnati Pediatric Rheumatology Tissue Repository (PRTR) (grant P30‐AR‐047363).
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Additional supplementary information cited in this article can be found online in the Supporting Information section
http://onlinelibrary.wiley.com/doi/10.1002/acr.25234
Author disclosures are available at
https://onlinelibrary.wiley.com/doi/10.1002/acr.25234
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Acquisition of data. Huang, Sompii-Montgomery, Patti, Pickering, Yasin, Do, Baker, Gao, Abdul-Aziz, Behrens, Canna, Clark, Co, Collins, Eberhard, Friedman, Graham, Hahn, Hersh, Hobday, Holland, Huggins, Li, Mannion, Manos, Neely, Onel, Orandi, Ramirez, Reinhard, Riskalla, Santiago, Stoll, Ting, Towe, Schulert.
All authors were involved in drafting the article or revising it critically for important intellectual content, and all authors approved the final version to be published. Dr. Schulert had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.
Study conception and design. Yasin, Grom, Towe, Schulert.
AUTHOR CONTRIBUTIONS
Analysis and interpretation of data. Huang, Sompii-Montgomery, Pickering, Do, Gao, Grom, Towe, Schulert.
ISSN:2151-464X
2151-4658
2151-4658
DOI:10.1002/acr.25234