Anti-glomerular basement membrane antibody disease: a rare autoimmune disorder affecting the kidney and the lung.

Abstract

Anti-glomerular basement membrane antibody disease is a rare, but well characterized cause of glomerulonephritis. By definition serum anti-GBM antibody and/or a linear binding of IgG detected by direct immunofluorescence (IF) in a histological specimen of the kidney or the lung have to be detected. These antibodies can lead to acute rapid progressive glomerulonephritis(RPGN) and/or pulmonary hemorrhage (PH) because of collagen similarities in the basement membrane. Principally anti-GBM antibody disease can be divided into two groups: anti-GBM antibody disease without PH was regarded as renal-limited anti-GBM antibody disease and that with PH was defined as Goodpasture's syndrome (GPS). The important determinant for the response of therapy and long term diagnosis on anti-GBM disease is early diagnosis to prevent endstage renal disease. Therefore, standard treatment is a combined therapy of plasmapherisis, prednisolone and cyclophosphamide. The aim of this review is an overview of the pathogenesis, clinical presentation, diagnosis and treatment of anti-GBM disease.

• Authors: Tobias Lahmer, Uwe Heemann
• Journal: Autoimmunity reviews (Autoimmun Rev) Vol. 12 Issue 2 Pg. 169-73 (Dec 2012) ISSN: 1873-0183 [Electronic] Netherlands
• PMID: 22546293 (Publication Type: Journal Article, Review)
• Copyright: Copyright © 2012. Published by Elsevier B.V.

Chemical References

• Autoantibodies
• antiglomerular basement membrane antibody

Topics

• Autoantibodies (immunology)
• Glomerulonephritis (diagnosis, epidemiology, etiology, therapy)
• Hemorrhage (diagnosis, epidemiology, etiology, therapy)
• Humans
• Lung Diseases (diagnosis, epidemiology, etiology, therapy)