We hypothesize that a peripheral blood biomarker or biological signature (gene or protein expression pattern) of idiopathic interstitial pneumonias (IIPs) will simplify and improve the accuracy of diagnosis of IIP and diagnose individuals at an earlier, more treatable, stage of their disease.

The Broad Challenge Area addressed in this proposal is (03) Biomarker Discovery and Validation, and the Specific Challenge Topic is 03-HL-101 (Identify and validate clinically relevant, quantifiable biomarkers of diagnostic and therapeutic responses for blood, vascular, cardiac, and respiratory tract dysfunction). Idiopathic interstitial pneumonia (IIP) is a lung disease(s) that primarily affects the elderly, but is present in all age groups. IIP causes respiratory insufficiency and is often fatal. In about half of the patients, the diagnosis requires an invasive lung biopsy which can cause complications, and is not always accurate.

The current diagnostic tools for IIP are inadequate. In addition to inaccurate diagnosis, they are very costly, and often result in delayed diagnosis and treatment. The challenge(s) we intend to address in this proposal is to improve the accurate and early diagnosis of idiopathic interstitial lung pneumonia (IIP), and to improve the ability to differentiate the subtypes of idiopathic interstitial pneumonias (IIPs) by developing peripheral blood biomarkers.

Inclusion Criteria:

• Sporadic cases of Idiopathic Pulmonary Fibrosis and other types of Idiopathic Interstitial Pneumonias.
• Family members ( with or without clinical disease) with a family history of pulmonary fibrosis.

Exclusion Criteria:

• None.

We are recruiting patients with Idiopathic Pulmonary Fibrosis and other types of Idiopathic Interstitial Pneumonias that occur sporadically or familial (2 or more affected individuals in a family).