Classification of Disease Process
Subjects are also classified according to the suspected underlying
biologic process(es) based on available clinical, genetic, radiologic,
and pathologic data. Reporting site investigators identified subjects as
having interstitial fibrosis (n=112), airway disease (n=198), pulmonary
hemorrhage (n=82), altered intrinsic lung development (n=104), pulmonary
alveolar proteinosis (n=30), immune deficiency or dysregulation (n=134),
or eosinophilic disorders (n=20). Among the 112 subjects with a fibrotic
disease process, 34 (30.4%) had connective tissue or immune-mediated
disorders and 25 (22.3%) had surfactant dysfunction. If an individual
experienced multiple co-existing disease processes, then each
contributing biologic mechanism was recorded. For example, 74% of
subjects characterized as having a fibrotic disease process were
designated to have an additional type of disease process present (e.g.,
immune dysregulation; airway disease).