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 interplay among the cells in the fibrotic niche with environmental factors such as extracellular matrix composition, topography and stiffness in addition to other factors are likely critical factors driving fibrotic progression. Unfortunately the composition of the fibroblastic focus is incompletely defined.
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