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Janus Kinase Inhibitors and Interstitial Lung Disease Associated With Pediatric Rheumatic Diseases: An Unexplored
Evdoxia Sapountzi1, Lampros Fotis2, Eleni Kotanidou1
12nd Department of Pediatrics, AHEPA University General Hospital, Aristotle University of Thessaloniki, Thessaloniki, GRC.
Insights
Janus kinase inhibitors (JAKis) show promise for rheumatic diseases with lung involvement. Further research is needed to confirm their efficacy and safety in pediatric patients with these complex conditions.
Area of Science:
- Rheumatology
- Pulmonology
- Pharmacology
Background:
- Rheumatic diseases frequently involve the lungs, leading to interstitial lung disease (ILD) and impacting patient survival.
- While biologics have improved outcomes, incomplete remission and adverse effects necessitate alternative treatments.
- Janus kinase inhibitors (JAKis) represent a novel therapeutic class with potential survival benefits.
Purpose of the Study:
- To review the rationale for using JAKis in rheumatic disease-associated ILDs.
- To summarize existing studies on JAKi efficacy in adult and pediatric patients with pulmonary involvement.
- To highlight the need for further research on JAKis in pediatric rheumatic lung disease.
Main Methods:
- Literature review of studies on JAKis in rheumatic diseases and ILDs.
- Focus on studies including adult and pediatric populations.
- Analysis of available data on efficacy and safety.
Main Results:
- JAKis are a promising therapeutic option for rheumatic disease-associated ILDs.
- Limited data exist on JAKi effectiveness in pediatric patients with pulmonary involvement.
- Current evidence suggests potential benefits but requires further investigation.
Conclusions:
- JAKis offer a potential survival benefit for patients with rheumatic diseases, including those with lung complications.
- More controlled, long-term studies are essential to establish the efficacy and safety of JAKis in pediatric rheumatic disease with lung involvement.
Abstract:
Rheumatic diseases are often complicated by lung disease, commonly presenting as interstitial lung disease (ILD), with potentially detrimental consequences for patient survival. Although less frequent in pediatric patients, pulmonary involvement may be observed in almost all childhood-onset rheumatic conditions. The development of biological disease-modifying anti-rheumatic drugs has significantly improved clinical outcomes. However, disease remission is not always complete or long-lasting, and treatment may need to be discontinued due to adverse effects. A novel class of drugs, namely Janus kinase inhibitors (JAKis), has been proposed to provide a significant survival benefit for patients with rheumatic diseases. Despite the ample literature on the efficacy and safety of JAKis in rheumatic disease, only a few studies have investigated the effectiveness of these drugs in patients with pulmonary involvement, and only two case reports have presented results in pediatric patients. We provide an overview of the rationale for using JAKis in ILDs associated with rheumatic disease and summarize the main studies evaluating their efficacy in both adult and pediatric patients. The present review highlights the need for controlled long-term studies to assess the efficacy and safety of JAKis in pediatric rheumatic disease complicated by lung disease.
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