End organ fibrosis accounts for up to 45% of deaths in developed countries. In particular, lung fibrosis is a devastating disease with poor prognosis. Despite development of two new drugs, their efficacy is still limited, highlighting the need to better understand the pathobiology that accounts for fibrotic disease progression in the presence and absence of acute exacerbation or infectious drivers.
Many lung diseases (IPF, COPD) are characterized by marked heterogeneity at the tissue level. Unfortunately, most of the tools we currently employ to understand lung disease are unable to elucidate the mechanisms that result in regional heterogeneity. Clinical studies and animal models, while invaluable, generally assume that all lung tissue is similarly affected based on the presence or absence of diagnostic criteria... more »
Our understanding of the factors driving IPF fibrotic progression remain incompletely understood. To develop effective therapies that arrest and reverse the fibrotic process, we must first identify the critical factors driving the spread of fibrosis. Fibroblastic foci, the sentinel morphologic lesion of IPF, are found at the advancing edge of fibrosis. Fibroblastic foci can be conceptualized as a fibrotic niche. The... more »