Fig. 4
From: Clinical, radiological and histopathological features of patients with familial pulmonary fibrosis
Histological images from a patient with a TERC variant (no 1). In the upper left lobe, there were separate subpleural or paraseptal areas similar to those of pleuroparenchymal fibroelastosis (A) with thick elastic fibers (B). There were also a few centrilobular areas with slightly fibrotic alveolar walls (C) and focal chronic inflammation (D), as well as peribronchiolar aggregations of fibromyxoid connective tissue and macrophages (E) and poorly formed granulomas (F). In the lower left lobe, fibrosis was more prominent, with centrilobular bridging fibrosis (G) and architectural remodeling (H) with occasional fibroblast foci (arrow)