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Updated: Apr 19, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Hydroxychloroquine in children with interstitial (diffuse parenchymal) lung diseases
Sarah Braun1, Marion Ferner, Kai Kronfeld
1Dr. von Hauner Children's University Hospital, Ludwig-Maximilians University, Munich, Germany; German Center for Lung Research (DZL), Munich, Germany.
Insights
Hydroxychloroquine (HCQ) may be safe for treating childhood interstitial lung disease (chILD), but more prospective studies are needed. Current evidence is based on limited case reports, highlighting the need for systematic data collection.
Area of Science:
- Pediatric Pulmonology
- Rheumatology
- Clinical Pharmacology
Background:
- Hydroxychloroquine (HCQ) is frequently used off-label for childhood interstitial lung disease (chILD).
- Limited studies exist on the efficacy and safety of HCQ in pediatric patients.
- A literature review was conducted to assess existing data on HCQ and chloroquine (CQ) in chILD.
Purpose of the Study:
- To review the literature on the use of chloroquine (CQ) and hydroxychloroquine (HCQ) in children with interstitial lung disease (chILD).
- To evaluate the reported efficacy and safety of HCQ in chILD based on available case reports and series.
Main Methods:
- Literature search of case reports and small series from 1984 to 2013.
- Identified 85 cases of chILD treated with CQ or HCQ, often with other medications.
- Analyzed reported outcomes, dosages, and side effects.
Main Results:
- A favorable response to HCQ or CQ was reported in 35 cases; outcomes were unclear or negative in others.
- HCQ dosage ranged from 5 to 10 mg/kg/day; no pharmacokinetic data exists.
- Gastrointestinal symptoms were common side effects; retinal changes were reported with CQ but not HCQ.
Conclusions:
- Based on retrospective data, HCQ use in chILD appears potentially safe, though bias is likely.
- Prospective data on HCQ efficacy and safety in chILD is lacking and urgently needed.
- Web-based registers and controlled investigations are recommended for systematic data collection.
Abstract:
Hydroxychloroquine (HCQ) is one of the drugs frequently used for the treatment of interstitial lung disease (ILD) in children (chILD). This use is off-label and studies to analyze the effect and safety of HCQ in chILD are lacking. Therefore, a literature research on the usage of chloroquine (CQ) and HCQ in these conditions was done. Eighty-five case reports and small series in the period from 1984 to 2013 were identified in which children with different diagnoses of ILD were treated with CQ or HCQ, sometimes in combination with other medication including steroids. A favorable response to HCQ or CQ was reported in 35 cases, whereas in the other cases the effect was negative or not clear. The dose of HCQ used was between 5 and 10 mg/kg body weight/day (bw/d). No pharmacokinetic studies have been done. The side effect profile in children seemed to be similar to that in adults. Most often gastrointestinal symptoms were reported. Three patients were found developing retinal changes during the treatment with CQ, whereas in none of the patients treated with HCQ retinal changes were reported. Based on retrospective case reports and small series likely to be reported with bias, the use of HCQ in chILD might be classified as safe. As no prospective data on efficacy and safety of HCQ in chILD are available, systematic collection is necessary. This may be achieved by web-based registers like the European Management Platform for Childhood Interstitial Lung Diseases. Prospective and controlled investigations of HCQ in patients with chILD are mandatory.
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