title: Diffuse and interstitial lung disease and childhood rheumatologic disorders.
abstract: OBJECTIVE
  Advances in genetics and clinical diagnostics, along with recently described clinical entities and refined classification schemes, have improved our understanding of diffuse and interstitial lung diseases in children. This review presents recent updates in these disorders in the context of systemic inflammatory conditions.
  
  
  RESULTS
  Classification of childhood diffuse lung disease (DLD) using adult paradigms is not useful. Distinct clinical-pathologic entities exist in children. Infants are more likely to present with genetic and developmental disorders, and older children with inflammatory and immune-mediated conditions. A combination of clinical evaluation, high-resolution computed tomography scanning, pulmonary function testing and serology, with bronchoscopy and surgical lung biopsy in selected cases, is most useful in the evaluation of DLD in the context of rheumatologic conditions. Common causes of DLD, such as infection, especially in the setting of immunodeficiency, must be ruled out. Optimal therapy for specific disorders will require careful analysis of data from national registries. Emerging use of biomarkers and high-throughput molecular analysis will yield novel insight into these disorders.
  
  
  CONCLUSIONS
  In the setting of known or suspected rheumatologic disorders, diagnosis and management of DLD are challenging, and require close collaboration among rheumatologists, pulmonologists, and other specialists.
authors: Division of Respiratory Medicine, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors: Sharon
authors: S
authors: Dell
authors: Matejka
authors: M
authors: Cernelc-Kohan
authors: James S
authors: JS
authors: Hagood
mesh_terms: D015415:Biomarkers / Q000378:metabolism; D002648:Child; D003240:Connective Tissue Diseases / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D003937:Diagnosis, Differential; D003948:Diagnostic Techniques, Respiratory System; D006801:Humans; D008171:Lung Diseases / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D012216:Rheumatic Diseases / Q000175:diagnosis / Q000209:etiology / Q000628:therapy
chemical_list: D015415:Biomarkers