Response to treatment and outcome can be evaluated in children based on several criteria such as decrease in cough and dyspnea, increase in oxygenation at rest and sleep, and changes in pulmonary function tests [1, 11] . Improvement on thoracic HRCT may also be seen, but tends to occur over a much longer period of time. Reports in pediatric ILD have not shown a good correlation between histological findings and outcome. Some children with relatively severe fibrosis on lung biopsy make good progress, whereas others with mild desquamation have a poor outcome. This is probably due to the variable severity of the disease in different parts of the lung especially in relation to the particular area biopsied, despite HRCT guidance. Overall a favorable response to corticosteroid therapy can be expected in 40-65% of cases, although significant sequelae such as limited exercise tolerance or the need for long-term oxygen therapy are often observed. Reported mortality rates are around 15%. The outcome for infants is more variable [1, 61] .