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Published on: June 16, 2020
Diagnosis and Management of the Systemic Juvenile Idiopathic Arthritis Patient with Emerging Lung Disease
Christopher Towe1,2, Alexei A Grom3,2, Grant S Schulert4,5
1Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Insights
Systemic juvenile idiopathic arthritis-associated chronic lung disease (SJIA-LD) is a serious complication. This review clarifies diagnosis, management, and emerging treatments for SJIA-LD, highlighting research needs.
Area of Science:
- Pediatric Rheumatology
- Pulmonology
- Immunology
Background:
- Chronic lung disease (CLD) is an emerging, severe complication in children with systemic juvenile idiopathic arthritis (SJIA-LD).
- Optimal diagnosis and management strategies for SJIA-LD are not yet established.
- Current understanding includes potential roles for biologic therapies and drug reactions with eosinophilia and systemic symptoms (DRESS).
Approach:
- This review synthesizes current knowledge on SJIA-LD's clinical spectrum and immunologic characteristics.
- A proposed algorithm for early detection and risk stratification of SJIA patients is discussed.
- Evaluation and treatment approaches, including pharmacologic and non-pharmacologic options, are examined.
Key Points:
- Biologic therapy's role in SJIA-LD pathogenesis and DRESS are considered.
- Diagnostic evaluation includes a risk-stratification algorithm for early LD detection.
- Treatment options encompass lymphocyte-targeting therapies, JAK inhibitors, and novel agents targeting IL-18 and IFNγ.
Conclusions:
- Further research is urgently needed to elucidate SJIA-LD mechanisms.
- Improved clinical research is essential for better disease detection and patient outcomes.
- Addressing unmet needs in basic discovery and clinical research will advance the care of children with SJIA-LD.
Abstract:
Chronic lung disease in children with systemic juvenile idiopathic arthritis (SJIA-LD) is an emerging and potentially life-threatening disease complication. Despite recent descriptions of its clinical spectrum, preliminary immunologic characterization, and proposed hypotheses regaarding etiology, optimal approaches to diagnosis and management remain unclear. Here, we review the current clinical understanding of SJIA-LD, including the potential role of biologic therapy in disease pathogenesis, as well as the possibility of drug reactions with eosinophilia and systemic symptoms (DRESS). We discuss approaches to evaluation of children with suspected SJIA-LD, including a proposed algorithm to risk-stratify all SJIA patients for screening to detect LD early. We review potential pharmacologic and non-pharmacologic treatment approaches that have been reported for SJIA-LD or utilized in interstitial lung diseases associated with other rheumatic diseases. This includes lymphocyte-targeting therapies, JAK inhibitors, and emerging therapies against IL-18 and IFNγ. Finally, we consider urgent unmet needs in this area including in basic discovery of disease mechanisms and clinical research to improve disease detection and patient outcomes.
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