Seven children aged 3 months to 11 years with histologically confirmed
interstitial lung disease (ILD) [6 with desquamative interstitial pne
umonitis (DIP) and I with chronic interstitial pneumonitis] were treat
ed with chloroquine, 10 mg/kg/day. One patient, diagnosed late in the
course of the disease, died after three weeks of treatment, despite th
e addition of systemic corticosteroids. Another patient responded to c
ombined therapy with chloroquine and prednisone and had a normal lung
biopsy after 6 months of treatment. He underwent surgical repair of mi
tral valve stenosis and died after extensive brain infarction. The oth
er 5 patients responded well to chloroquine therapy with major improve
ment in oxygenation within a few weeks and in lung function over the n
ext few months. They remained well clinically and physiologically, inc
luding 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 patient
s has developed retinopathy or any other ocular complication. Bronchoa
lveolar ravage was a useful tool for evaluation of the activity of the
disease (predominance of neutrophils) in 3 out of 4 patients. We sugg
est 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. (C)
1994 Wiley-Liss, Inc.