Two Inherited Forms of Pulmonary Fibrosis and Their Immunome

Incomplete definitions exist for the immunome (serum cytokine profiling, immune cell phenotype, and gene expression) in pulmonary fibrosis. Research on inherited types of pulmonary fibrosis sheds light on the general mechanisms underlying fibrotic lung disease. Highdimensional flow cytometry and large-scale gene expression of peripheral blood mononuclear cells were performed and compared in a cohort with Familial Pulmonary Fibrosis (FPF), an autosomal dominant disorder with incomplete penetrance; Hermansky-Pudlak Syndrome Pulmonary Fibrosis (HPSPF), a rare autosomal recessive disorder; and their unaffected relatives in order to define the cellular and molecular immunologic phenotype in According to our findings, patients with FPF had elevated peripheral blood levels of activated central memory helper cells. Patients who had either familial or HPSPF antibodies had higher proportions of CD38+ memory CD27+ B-cells, IgA+ memory CD27+ B-cells, IgM+ and IgD+ B-cells, and CD39+ T helper cells, whereas lower proportions of CD39 T helper cells were present. In addition to changed levels of various analytes, such as leptin, cytokines, and growth factors, gene expression and serum proteomic studies of circulating mononuclear cells revealed enrichment of upregulated genes linked to mitosis and cell cycle control. The extra-pulmonary immune system's dysregulation is a phenotypic trait of FPF or HPSPF, to sum up. To understand the role of immune system dysregulation in the aetiology of pulmonary fibrosis, more research on the blood immunome is recommended