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Diagnosis and Management of the Systemic Juvenile Idiopathic Arthritis Patient with Emerging Lung Disease

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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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Acknowledgements

We thank Dr Kathryn Wikenheiser-Brokamp for providing lung biopsy images shown in Fig. 1.

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Authors and Affiliations

  1. Division of Pulmonary Medicine, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA

    Christopher Towe

  2. Division of Rheumatology, Cincinnati Children’s Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, 3333 Burnet Ave, MLC 4010, Cincinnati, OH, 45229, USA

    Alexei A. Grom & Grant S. Schulert

  3. Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA

    Christopher Towe, Alexei A. Grom & Grant S. Schulert

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Dr Towe received consulting fees from Boehringer Ingelheim. Dr Grom reports consulting fees and research support from Novartis, SOBI, and AB2Bio. Dr Schulert has received consulting fees from SOBI and research support from IpiNovyx.

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Dr Schulert developed initial outline. All authors wrote sections of the first draft. All authors reviewed and approved final version.

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Towe, C., Grom, A.A. & Schulert, G.S. Diagnosis and Management of the Systemic Juvenile Idiopathic Arthritis Patient with Emerging Lung Disease. Pediatr Drugs 25, 649–658 (2023). https://doi.org/10.1007/s40272-023-00593-8

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