Pharmacological strategies in the idiopatic pulmonary fibrosis of children

Idiopathic pulmonary fibrosis is characterized by radiographically evident interstitial infiltrates and progressive dyspnea with rapid decline of overall lung functions. The clinical course of this disease is highly variable; however, its prognosis remains poor without adequate and prompt therapeutical support. Due to the fact that barely a quarter of patients responds to steroids alone, cytostatics are often prescribed simultaneously with low dose corticosteroids in the pediatric age too. Cytokine or anti-cytokine therapy and the role of new antifibrotic drugs are still at the experimental stage of definition and will be discussed in the following review.