バイオテロとバイオディフェンスのジャーナル

抽象的な

Bioterrorism agent and new tool for Monogenic Auto inflammatory Disorders

Yang Hoe

The discovery that several of these conditions are caused by mutations in proteins involved in the mechanisms of innate immune response, including components of the inflammasome, cytokine receptors, and receptor antagonisin,has revolutionised the treatment of monogenic auto inflammatory disorders, an expanding group of hereditary diseases characterised by apparently unprovoked recurrent episodes of inflammation, without high-titre autoantibodies or antigen-specific T cells. The purpose of this review is to summarise recent research and experience regarding the therapeutic use of biologic medications in paediatric and adult patients with monogenic auto inflammatory diseases.

The stages of frozen shoulder, which depict the progression of processes from capsular inflammation and fibrosis to spontaneous resolution of this fibrosis, are largely understood. The underlying pathophysiologic mechanism,however, is still poorly understood. The treatment of frozen shoulder is still debatable due to this. A crucial step in the creation of a novel treatment for people with frozen shoulder is figuring out the pathophysiological causes of the condition. The basic pathophysiology of frozen shoulder is reviewed in this article along with what is currently known about it. Despite conflicting and ambiguous findings, papers on the pathophysiology of frozen shoulder have shown that cytokines, growth factors, matrix metalloproteinase, and immune cells are involved in both inflammation and fibrosis. Fibroblast activity is governed by proinflammatory cytokines and growth factors generated by immune cells,while matrix remodelling is controlled by matrix metalloproteinase and their inhibitors. The biology of these processes at specific stages needs to be better characterised in order to increase our understanding of the disease continuum. To more precisely define the function of cytokines, growth factors, matrix metalloproteinase, and immune cells, additional fundamental investigations utilising standardised protocols are necessary. The findings of these investigations will shed much-needed light on the pathogenesis of frozen shoulder and aid in the discovery of novel therapeutic targets.