Pulmonary vascular disorders masquerading as interstitial lung disease

• Published in: Pediatric pulmonology Vol. 20; no. 5; p. 284
• Main Authors: Sondheimer, H M, Lung, M C, Brugman, S M, Ikle, D N, Fan, L L, White, C W
• Format: Journal Article
• Language: English
• Published: United States 01-11-1995
• Subjects: 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
• ISSN: 8755-6863
• 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.