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. 2024 Jan;26(1):19-30.
doi: 10.1007/s40272-023-00603-9. Epub 2023 Nov 10.

Emerging Treatments for Childhood Interstitial Lung Disease

Nicol Bernardinello  1 , Matthias Griese  2 , Raphaël Borie  3 , Paolo Spagnolo  4
Affiliations

Emerging Treatments for Childhood Interstitial Lung Disease

Nicol Bernardinello et al. Paediatr Drugs. 2024 Jan.

Abstract

Childhood interstitial lung disease (chILD) is a large and heterogeneous group of disorders characterized by diffuse lung parenchymal markings on chest imaging and clinical signs such as dyspnea and hypoxemia from functional impairment. While some children already present in the neonatal period with interstitial lung disease (ILD), others develop ILD during their childhood and adolescence. A timely and accurate diagnosis is essential to gauge treatment and improve prognosis. Supportive care can reduce symptoms and positively influence patients' quality of life; however, there is no cure for many of the chILDs. Current therapeutic options include anti-inflammatory or immunosuppressive drugs. Due to the rarity of the conditions and paucity of research in this field, most treatments are empirical and based on case series, and less than a handful of small, randomized trials have been conducted thus far. A trial on hydroxychloroquine yielded good safety but a much smaller effect size than anticipated. A trial in fibrotic disease with the multitargeted tyrosine kinase inhibitor nintedanib showed similar pharmacokinetics and safety as in adults. The unmet need for the treatment of chILDs remains high. This article summarizes current treatments and explores potential therapeutic options for patients suffering from chILD.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Fig. 1
Fig. 1
Seventeen-year-old girl with SP-C deficiency and interstitial lung disease. CT scans show extensive ground glass opacity and traction bronchiectasis throughout both lungs. CT computed tomography, SP-C surfactant protein C
Fig. 2
Fig. 2
Seven-year-old boy ABCA3-related interstitial lung disease. CT scans show bilateral ground glass opacity. CT computed tomography
See this image and copyright information in PMC

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