Immune‐Mediated Diseases Involving Basement Membranes

Abstract

Publisher Summary This chapter describes the three types of immunologically mediated renal diseases from the primary glomerular disease group: antiglomerular basement membrane disease (Goodpasture syndrome), membranous nephropathy, and postinfectious glomerulonephritis. There are primary and secondary glomerular diseases that ultimately may affect the glomerular and tubular basement membranes. Notable examples of the former group are postinfectious glomerulonephritis, immunoglobulin A nephropathy, antiglomerular basement membrane disease, and membranous nephropathy. The chapter discusses the immunization of animals with heterologous glomerulus‐rich antigens in which an autologous response to glomerular basement membrane (GBM) antigens is involved in pathogenesis. The discovery of proteoglycans, perlecan, and agrin in basement membranes complete the cycle of the search for their antigenic components. A comparative study between proteoglycans isolated from the bovine lens capsule and the GBM indicates partial immunologic cross reactivity of these molecules with each other and with perlecan from the murine Engelbreth–Holm–Swarm (EHS) tumor. The search for the nature of the antigenic components of basement membranes that may be responsible for the renal pathology involving these structures produce a series of studies that culminated in the elucidation of the biochemical basis of at least one disorder—namely, Good–pasture syndrome.