[Pulmonary fibrosis in children: diagnosis and therapy]

Pulmonary fibrosis is a chronic disorder characterized by inflammation, injury and fibrosis of the alveolar structures and consequent loss of functional alveolar-capillary units. It involves a number of unresolved terminologic, pathogenetic, diagnostic and therapeutic controversies. The key to understanding the disease is the recognition that inflammation is the first event. The staging of the patients, important for correct therapy decision, is made using methods that specifically evaluate the inflammation such as open lung biopsy, bronchoalveolar lavage, and Gallium 67 scanning. The most rational therapeutic approach is to suppress the inflammation and to prevent further injury and scarring. The drugs of choice for treatment are corticosteroids (prednisone particularly). It is suggested to use large doses during the initial portion of the therapeutic trial followed by a tapering of the dose. If the patient does not respond to corticosteroids other immunosuppressive agents can be considered, particularly cyclophosphamide. The frequency and duration of response to these regimens remains to be defined.