IgG4-related lung disease manifested as four major categories of CT features. Pathologically, these features corresponded to IgG4-related sclerosing inflammation along the intrapulmonary connective tissue.
Background: Fractalkine expressed on endothelial cells mediates activation and adhesion of leucocytes expressing its receptor, CX 3 CR1. Soluble fractalkine exhibits chemotactic activity for leucocytes expressing CX 3 CR1. Objective: To determine the role of fractalkine and its receptor in systemic sclerosis (SSc) by assessing their expression levels in patients with this disease. Methods: The expression of fractalkine and CX 3 CR1 in the skin and lung tissues was immunohistochemically examined. Circulating soluble fractalkine levels were examined by enzyme linked immunosorbent assay (ELISA). Blood samples from patients with SSc were stained for CX 3 CR1 with flow cytometric analysis. Results: CX 3 CR1 levels on peripheral monocytes/macrophages and T cells were found to be raised in patients with diffuse cutaneous SSc. The numbers of cells expressing CX 3 CR1, including monocytes/ macrophages, were increased in the lesional skin and lung tissues from patients with diffuse cutaneous SSc. Fractalkine was strongly expressed on endothelial cells in the affected skin and lung tissues. Soluble fractalkine levels were significantly raised in sera and were associated with raised erythrocyte sedimentation rates, digital ischaemia, and severity of pulmonary fibrosis. Conclusions: Up regulated expression of fractalkine and CX 3 CR1 cooperatively augments the recruitment of mononuclear cells expressing CX 3 CR1 into the affected tissue of SSc, leading to inflammation and vascular injury.
The characteristic CT findings of patients with anti-ARS-ILD were areas of ground-glass attenuation and reticulation, predominantly distributed as lower and peribronchovascular lesions, which is compatible with NSIP. One-third of patients showed OP with fibrosis.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.