# Interstitial Lung Abnormalities: Defining the Phenotype, Causes, and Consequences.

> **NIH NIH R01** · BRIGHAM AND WOMEN'S HOSPITAL · 2020 · $866,907

## Abstract

7. Project Summary/Abstract
The primary objective of this proposal is to provide a more precise characterization of
the factors that predict pulmonary fibrosis (PF) development and progression in specific
populations with the goal of defining groups that might benefit from participation in early
PF screening studies. Idiopathic pulmonary fibrosis (IPF), the most common and severe
form of pulmonary fibrosis (PF) has a mortality rate comparable to that of many end-
stage malignancies Although IPF has historically been unresponsive to
pharmacotherapy, recent studies have demonstrated that medical therapy can reduce
the rate of decline in lung function, particularly when started early in the course of
disease. Our recent findings demonstrated that early disease detection for PF is an
achievable goal. In 1st degree relatives of patients with PF we have demonstrated ILA in
38% of those we have evaluated, and 33% of those with ILA were found to have signs of
more advanced disease. These relatives have been referred for clinical evaluations,
some of whom have begun on anti-fibrotic therapy for IPF/familial PF. While these
findings demonstrate that a landmark shift from reacting - to preventing – PF progression
in close relatives is possible, it is not known the extent to which early PF can be detected
in other unique populations at risk. Based on these findings we hypothesize that
measurable characteristics can be identified in 1) smokers with and without COPD, 2) in
patients with early stage lung cancer, and 3) in those with prior imaging abnormalities,
that will help to distinguish those who already have PF (and those with the greatest risk
to progress from early stages of PF) from those unlikely to develop this disease. The
results of these studies will improve our understanding of early disease detection for PF,
as well as setting the stage for trials aimed at the recruiting these specific populations for
early institution of novel and existing medical therapies.

## Key facts

- **NIH application ID:** 9890852
- **Project number:** 5R01HL111024-07
- **Recipient organization:** BRIGHAM AND WOMEN'S HOSPITAL
- **Principal Investigator:** GARY MATTHEW HUNNINGHAKE
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2020
- **Award amount:** $866,907
- **Award type:** 5
- **Project period:** 2013-07-01 → 2023-04-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/9890852

## Citation

> US National Institutes of Health, RePORTER application 9890852, Interstitial Lung Abnormalities: Defining the Phenotype, Causes, and Consequences. (5R01HL111024-07). Retrieved via AI Analytics 2026-05-23 from https://api.ai-analytics.org/grant/nih/9890852. Licensed CC0.

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