University of Virginia Natural History Study

A Study of the Natural Progression of Interstitial Lung Disease

Data and specimens will be collected longitudinally from patients seen in the UVA Interstitial Lung Disease (ILD) clinic in order to describe the phenotypic expression of various interstitial lung diseases. Samples will also be collected from a control group for comparison purposes.

All data will be entered into a repository for future research purposes or screening for new studies that become available. This data will help identify trends and hopefully lead to a better understanding of the disease progression, treatment options, and outcomes.

Following the clinical course of patients with ILD will allow description of the natural history of these diseases and prospective analysis of the following specific questions:

1. Can surrogate physiological markers of disease progression be used in place of mortality? Mortality of a defined cohort of patients will be developed. The surrogate markers include but are not limited to serial cardiopulmonary exercise testing, pulmonary function testing, 6-minute walk testing, HRCT scanning and echocardiography.
2. Do alternative assessments such as quality of life (QOL) questionnaires provide early prediction of physiological change as measured by the previously described parameters? Periodic QOL questionnaires are regularly conducted in clinic and will be followed
3. Does rate of deterioration as measured by these previously described parameters affect response to therapy? If we pre-identify rapid decliners from stable subjects, does this variable affect the response to whatever therapy is applied?
4. Can genetic analysis, genomics, proteomics, microbial and other biomarkers in the blood and cheeks provide insight into the polymorphisms and other elements related to the etiology and pathology of the lung damage?

Inclusion Criteria:

• Patient presenting to University of Virginia ILD or Pulmonary clinic
• Family members accompanying patients (as control subjects)

Exclusion Criteria:

• Control subjects cannot have ILD