| H01721 | |
| H number | H01721 |
| Name | Anti-glomerular basement membrane (GBM) disease; Goodpasture syndrome |
| Description | Goodpasture syndrome (GS), or anti-glomerular basement membrane (anti-GBM) disease, is a rare and organ-specific autoimmune disease defined by anti-GBM antibody-mediated damage (mainly immunoglobulin G-1) resulting in progressive crescentic glomerulonephritis and, frequently, diffuse pulmonary alveolar hemorrhage. Clinically, GS is characterized by pulmonary hemorrhage and renal failure, although the clinical spectrum may range from only mild symptoms to a relentless and finally lethal outcome. This disease accounts for 10-20% of the patients with acute renal failure following a rapidly progressive glomerulonephritis. The disease is caused by an autoimmune response against the non-collagenase domain of the alpha 3 chain of type IV collagen [alpha3(IV)NC1], found in the glomerular and alveolar basement membranes. Without prompt diagnosis and treatment, the disease can lead to bleeding in the lungs, kidney failure, and even death. Early and intensive treatment with plasmapheresis and immunosuppression with systemic corticosteroids pending results of diagnostic testing, and later cyclophosphamide, is often beneficial, with 90% of patients surviving the acute presentation of GS. |
| Category | Immune system disease |
| Network | - |
| Gene | - |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | - |
| Comment | Autoantigen: COL4A3 [HSA:1285] [KO:K06237] See also H01688 Rapidly progressive glomerulonephritis (RPGN). |
| Other DBs | ICD-11: MF85 ICD-10: M31.0 N08.5 MeSH: D019867 |
| Reference | PMID:18502098 AUTHORS Ooi JD, Holdsworth SR, Kitching AR TITLE Advances in the pathogenesis of Goodpasture's disease: from epitopes to autoantibodies to effector T cells. JOURNAL J Autoimmun 31:295-300 (2008) DOI:10.1016/j.jaut.2008.04.005 PMID:21207195 AUTHORS Chan AL, Louie S, Leslie KO, Juarez MM, Albertson TE TITLE Cutting edge issues in Goodpasture's disease. JOURNAL Clin Rev Allergy Immunol 41:151-62 (2011) DOI:10.1007/s12016-010-8222-2 PMID:23806563 AUTHORS Dammacco F, Battaglia S, Gesualdo L, Racanelli V TITLE Goodpasture's disease: a report of ten cases and a review of the literature. JOURNAL Autoimmun Rev 12:1101-8 (2013) DOI:10.1016/j.autrev.2013.06.014 PMID:25462583 AUTHORS Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Pagliuca G, Martellucci S, Turchetta R, Longo L, De Vincentiis M TITLE Goodpasture's syndrome: a clinical update. JOURNAL Autoimmun Rev 14:246-53 (2015) DOI:10.1016/j.autrev.2014.11.006 |