Paediatric interstitial lung disease (PILD)-An update

Interstitial lung disease affects approximately 1 in 100,000 infants and children. The aetiology of as many as 40-50% of cases remains unknown at present. Recent advances in the understanding of surfactant genetics and the description of specific ultrastructural anomalies have advanced our knowledge of the underlying pathophysiology and the genetics of familial cases. Investigations should aim to exclude known underlying causes. A computed tomography (CT) examination is useful in assessing parenchymal involvement, but the lung biopsy remains most useful when the aetiology is unclear. There are no controlled trials of treatment because of the relatively small numbers of patients involved. Sixty-five percent of patients are likely to respond well to corticosteroids with or without hydroxychloroquine. About 10-15% will need continuing treatment over many months or years. The mortality rate among those with idiopathic disease is 15%.