INTERSTITIAL LUNG-DISEASE IN CHILDREN - AN ORPHAN LUNG-DISEASE

Interstitial lung disease (ILD) in children represents a heterogeneous group of disorders that result from inflammation of the pulmonary int erstitium and perialveolar tissues and share common histologic, radiol ogic, and clinical characteristics. The histologic marker of ILD, thic kening of the alveolar wall, result from responses of the lung to inju ry from known and unknown causes with recruitment of inflammatory and immunoregulatory cells. The inflammatory responses in the pulmonary in terstitium can be completely or partially reversible or can progress t o fibrosis and/or to irreversible ''end-stage lung'' disease. Unlike a sthma, another inflammatory disease of the respiratory system, ILD in children has not attracted enough interest or financial support for cr itical scientific efforts to advance the knowledge of this ''orphan lu ng disease.'' This review emphasizes the pathogenesis, classification, diagnostic evaluation, and general principles of monitoring and treat ment.