Interstitial Lung Disease in Children: An Orphan Lung Disease

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Abstract
Interstitial lung disease (ILD) in children represents a heterogeneous group of disorders that result from inflammation of the pulmonary interstitium and perialveolar tissues and share common histologic, radiologic, and clinical characteristics. The histologic marker of ILD, thickening of the alveolar wall, result from responses of the lung to injury from known and unknown causes with recruitment of inflammatory and immunoregulatory cells. The inflammatory responses in the pulmonary interstitium can be completely or partially reversible or can progress to flbrosis and/or to irreversible "end-stage lung" disease. Unlike asthma, another inflammatory disease of the respiratory system, ILD in children has not attracted enough interest or financial support for critical scientific efforts to advance the knowledge of this "orphan lung disease." This review emphasizes the pathogenesis, classification, diagnostic evaluation, and general principles of monitoring and treatment.