Some Aspects of Inborn and Acquired connective Tissue Diseases: A Special Emphasis on Renal Disease

摘要: Publisher Summary The complex interplays between extracellular matrix (ECM) and the immune system represent a particular area of intense clinical research. This chapter demonstrates how is involved in acquired diseases. focuses on renal Indeed, multiple pathogenetic mechanisms involving glomeruli, interstitium or both lead to ECM accumulation dysfunction. During glomerular expansion, specialized cellular structures are replaced by fibroblasts, collagen mesenchymal matrix, with concomitant disruptions kidney's functions filtration, secretion reabsorption. pathogenesis involves different types cells various growth factors inflammatory released resident infiltrating such as T cells, neutrophils, monocytes/macrophages platelets. On other hand, autoimmune attack against components can also result severe disorders. analyzes specific involvement biochemically defined inborn diseases emphasis immune-mediated injury. describes collagenrelated disorders special attention kidney. provides brief description normal structure considers two fields pathology which plays central role: (1) expansion (2) target for autoantibodies.