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Impact of pulmonary vascular volume on mortality in IPF: is it time to reconsider the role of vasculature in disease pathogenesis and progression?

We were pleased to read the letter from E. Puxeddu and co-workers, written in response to our observation that an increased pulmonary vessel volume, derived using quantitative computed tomography (CT) analysis, strongly predicts mortality in patients with idiopathic pulmonary fibrosis (IPF) [1]. E. Puxeddu and his team highlight a selection of studies that have identified pulmonary vascular alterations in IPF, similar to those seen in pulmonary venous occlusive disease (PVOD) and pulmonary capillary haemangiomatosis (PCH) [2–4]. They suggest that when considered in the light of these previous reports, our findings add support to the idea that vascular alterations might be the first pathological lesion in IPF preceding, and leading to, fibrogenesis. Understanding the complexities of the pulmonary vasculature is an important but neglected field of study in IPF http://ow.ly/anqV307U5rA