title: Absence of skin rash in Goodpasture's syndrome: the hyaluronan effect.
abstract: Goodpasture's syndrome is a rare disease that involves rapidly progressive kidney failure as well as hemorrhagic lung disease. It is a form of autoimmune disorder with unusual features; marked male preponderance, in contrast with other autoimmune disease in which females are at far greater risk. The autoantibodies are directed again the carboxy-extension non-collagenous (NC1) portion of one of the basement membrane-specific collagen IV alpha 3 chains. Basal laminas throughout the body share this structure, including those in kidney, lung and skin. But curiously, skin is rarely involved in Goodpasture's syndrome. Hyaluronan is a large extracellular matrix carbohydrate polymer. Half of total body hyaluronan occurs in skin. High molecular weight hyaluronan, a potent immunosuppressive polymer, might be functioning as an immune shield for skin in Goodpasture's syndrome, and be the basis for the anomaly. A summary of this putative effect is described, including possible molecular mechanisms involved, and suggestions for testing this hypothesis.
authors.affiliation: Division of Nephrology, Elmhurst Hospital Center, Icahn School of Medicine at Mount Sinai, 79-01 Broadway, Elmhurst, NY 11373, United States. Electronic address: Sterna@nychhc.org.
authors.forename: A
authors.initials: A
authors.lastname: Stern
authors.affiliation: Department of Basic Biomedical Sciences, Touro/Harlem College of Osteopathic Medicine, 230 West-125th Street, New York, NY 10027, United States.
authors.forename: R
authors.initials: R
authors.lastname: Stern
mesh_terms: D019867:Anti-Glomerular Basement Membrane Disease / Q000150:complications / Q000175:diagnosis*; D001323:Autoantibodies / Q000737:chemistry; D001485:Basement Membrane / Q000276:immunology; D002241:Carbohydrates / Q000737:chemistry; D003094:Collagen / Q000737:chemistry; D024141:Collagen Type IV / Q000276:immunology; D005076:Exanthema / Q000150:complications / Q000175:diagnosis*; D005109:Extracellular Matrix / Q000378:metabolism; D005260:Female; D006801:Humans; D006820:Hyaluronic Acid / Q000737:chemistry*; D007107:Immune System; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007678:Kidney Glomerulus / Q000276:immunology; D008297:Male; D008970:Molecular Weight; D011108:Polymers / Q000737:chemistry; D012867:Skin / Q000276:immunology
chemical_list: D001323:Autoantibodies; D002241:Carbohydrates; D024141:Collagen Type IV; D007166:Immunosuppressive Agents; D011108:Polymers; D006820:Hyaluronic Acid; D003094:Collagen