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Chloroquine treatment of interstitial lung disease in children
A Avital1, S Godfrey, C Maayan
1Institute of Pulmonology, Hadassah University Hospital, Jerusalem, Israel.
Insights
Chloroquine effectively treats interstitial lung disease (ILD) in children, improving oxygenation and lung function. Long-term follow-up showed sustained clinical benefit without ocular complications, suggesting chloroquine as a viable treatment option.
Area of Science:
- Pediatric Pulmonology
- Pharmacology
- Rheumatology
Background:
- Interstitial lung disease (ILD) in children is a group of rare, serious lung disorders.
- Limited effective long-term treatment options exist for pediatric ILD, particularly for desquamative interstitial pneumonitis (DIP).
Purpose of the Study:
- To evaluate the efficacy and safety of chloroquine as a treatment for histologically confirmed ILD in children.
- To assess long-term clinical and physiological outcomes in pediatric patients treated with chloroquine.
Main Methods:
- Seven children (3 months to 11 years) with ILD (6 DIP, 1 chronic interstitial pneumonitis) received chloroquine (10 mg/kg/day).
- Clinical response, oxygenation, lung function, and incremental exercise tests were monitored.
- Bronchoalveolar lavage was used to assess disease activity in some patients.
- Ocular complications were specifically monitored throughout the follow-up period.
Main Results:
- Five of seven patients showed significant improvement in oxygenation and lung function within weeks to months of chloroquine treatment.
- These five patients maintained clinical and physiological stability for a mean follow-up of 9.8 years.
- No ocular complications, such as retinopathy, were observed in any patient.
- Bronchoalveolar lavage indicated disease activity (neutrophil predominance) in 3 of 4 evaluable patients.
Conclusions:
- Chloroquine demonstrates efficacy as a treatment for pediatric interstitial lung disease, including desquamative interstitial pneumonitis.
- Long-term administration of chloroquine in children with ILD appears safe, with no reported ocular side effects.
- Incremental exercise testing can be a valuable tool for monitoring treatment efficacy in pediatric ILD.
Abstract:
Seven children aged 3 months to 11 years with histologically confirmed interstitial lung disease (ILD) [6 with desquamative interstitial pneumonitis (DIP) and 1 with chronic interstitial pneumonitis] were treated with chloroquine, 10 mg/kg/day. One patient, diagnosed late in the course of the disease, died after three weeks of treatment, despite the addition of systemic corticosteroids. Another patient responded to combined therapy with chloroquine and prednisone and had a normal lung biopsy after 6 months of treatment. He underwent surgical repair of mitral valve stenosis and died after extensive brain infarction. The other 5 patients responded well to chloroquine therapy with major improvement in oxygenation within a few weeks and in lung function over the next few months. They remained well clinically and physiologically, including a normal response to incremental exercise, during a mean follow-up period of 9.8 years (range 3.5 to 15.7 years). None of the patients has developed retinopathy or any other ocular complication. Bronchoalveolar lavage was a useful tool for evaluation of the activity of the disease (predominance of neutrophils) in 3 out of 4 patients. We suggest that chloroquine should be considered as an effective treatment in ILD in children. Incremental exercise test may be helpful for routine follow-up and evaluation of the efficacy of a specific treatment.