Pulmonary vascular disorders masquerading as interstitial lung disease

Pediatr Pulmonol. 1995 Nov;20(5):284-8. doi: 10.1002/ppul.1950200505.


During the acquisition of a series of 92 children with interstitial lung disease (ILD) over a 14 year period, a significant minority (8/92 or 9%) were initially diagnosed as having ILD, but were subsequently found to have a variety of arterial, venous, and/or capillary disorders that explained their initial pulmonary findings. This subgroup of patients has had a very high morbidity and mortality, with only three of eight patients currently surviving. The presentation, evaluation, and natural history of these eight children were reviewed. We developed a strategy of cardiac and pulmonary evaluation for children presenting with clinical and radiographic features of ILD that helped us to identify rapidly those with pulmonary vascular disorders.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Cardiovascular Diseases / diagnosis*
  • Cardiovascular Diseases / physiopathology
  • Child
  • Chronic Disease
  • Diagnosis, Differential
  • Echocardiography
  • Electrocardiography
  • Humans
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / physiopathology
  • Incidence
  • Infant
  • Infant, Newborn
  • Logistic Models
  • Lung Diseases, Interstitial / diagnosis*
  • Lung Diseases, Interstitial / physiopathology
  • Pulmonary Veno-Occlusive Disease / diagnosis
  • Pulmonary Veno-Occlusive Disease / physiopathology
  • Retrospective Studies
  • Risk Factors
  • Survival Rate
  • Telangiectasia, Hereditary Hemorrhagic / diagnosis
  • Telangiectasia, Hereditary Hemorrhagic / physiopathology