An autopsy case of idiopathic pulmonary fibrosis with remarkable honeycomb cyst expansion

Abstract

Herein, we report an autopsy case of idiopathic pulmonary fibrosis (IPF) in which remarkable honeycomb cyst expansion appeared in the clinical course. Radiological findings initially showed subpleural predominant reticulation that had progressed to usual interstitial pneumonia with honeycomb cysts, along with a restrictive pattern in the pulmonary function tests. The diameter of honeycomb cysts had gradually increased, and some cysts had abruptly expanded at the end stage. Based on pathological findings of autopsy specimens, bronchiectasis, alveolar collapse due to inflammation, and check-valve mechanism caused by a slit-like orifice of the cysts could have contributed to honeycomb cyst expansion.

Cell-Specific Response of NSIP- and IPF-Derived Fibroblasts to the Modification of the Elasticity, Biological Properties, and 3D Architecture of the Substrate

“…On the nanoscopic scale, the subcellular mechanisms are mainly affected, while on the microscopic scale, the topography of the substrate impacts the whole cell behaviors, such as cell morphology [ 88 , 89 , 96 ]. For lung fibrosis, the homogeneous porous architecture of normal lung tissue changes due to the fibrotic process into a honeycomb pattern [ 53 , 62 , 97 , 98 ]; therefore, the topography on the microscopic scale could also be a factor that impacts the behavior of fibroblasts derived from IPF and NSIP differently. To study this issue, 3D PDMS substrates were prepared using two approaches, based on the addition of soluble particles to the PDMS mixture or their molding with a predefined master, and used as substrates for the culture of IPF- and NSIP-derived fibroblasts, as well as healthy fibroblasts.…”