anti glomerular basement membrane disease

Summary

Summary: An autoimmune disease of the KIDNEY and the LUNG. It is characterized by the presence of circulating autoantibodies targeting the epitopes in the non-collagenous domains of COLLAGEN TYPE IV in the basement membranes of kidney glomeruli (KIDNEY GLOMERULUS) and lung alveoli (PULMONARY ALVEOLI), and the subsequent destruction of these basement membranes. Clinical features include pulmonary alveolar hemorrhage and glomerulonephritis.

Top Publications

  1. Saus J, Wieslander J, Langeveld J, Quinones S, Hudson B. Identification of the Goodpasture antigen as the alpha 3(IV) chain of collagen IV. J Biol Chem. 1988;263:13374-80 pubmed
  2. Garnier P, Deprele C, Pilonchéry B, Michel D. [Cerebral angiitis and Goodpasture's syndrome]. Rev Neurol (Paris). 2003;159:68-70 pubmed
    ..Clinical cerebral manifestations in our case are probably du to a angiitis. Few cases are reported in the literature. The role of antiglomerular basement membrane antibody in the development of the cerebral angiitis is possible...
  3. Rydel J, Rodby R. An 18-year-old man with Goodpasture's syndrome and ANCA-negative central nervous system vasculitis. Am J Kidney Dis. 1998;31:345-9 pubmed
    ..From an extensive review of the literature, this is the first case of anti-neutrophil cytoplasmic antibody-negative central nervous system (CNS) vasculitis in a patient with Goodpasture's syndrome...
  4. Abbate M, Kalluri R, Corna D, Yamaguchi N, McCluskey R, Hudson B, et al. Experimental Goodpasture's syndrome in Wistar-Kyoto rats immunized with alpha3 chain of type IV collagen. Kidney Int. 1998;54:1550-61 pubmed
  5. Kalluri R. Goodpasture syndrome. Kidney Int. 1999;55:1120-2 pubmed
  6. Gittins N, Basu A, Eyre J, Gholkar A, Moghal N. Cerebral vasculitis in a teenager with Goodpasture's syndrome. Nephrol Dial Transplant. 2004;19:3168-71 pubmed
  7. Ohashi N, Sugiura T, Isozaki T, Yamamoto T, Hishida A. Anti-glomerular basement membrane antibody-induced glomerulonephritis with periglomerular granulomatous reaction and massive renal eosinophilic infiltration. Am J Kidney Dis. 2003;42:E28-35 pubmed
    ..He was discharged from our hospital 65 days after admission. Three months later, his anti-GBM antibody level was less than 10 EU, and the number of peripheral eosinophils stayed with the normal range...
  8. Andrassy M, Sis J, Waldherr R, Zeier M, Schwenger V. The patient with C-ANCA/PR3-ANCA-positive crescentic pauci-immune glomerulonephritis and recurrence of nephritic sediment. Nephrol Dial Transplant. 2008;23:2084-7 pubmed publisher
  9. Aki K, Shimizu A, Masuda Y, Kuwahara N, Arai T, Ishikawa A, et al. ANG II receptor blockade enhances anti-inflammatory macrophages in anti-glomerular basement membrane glomerulonephritis. Am J Physiol Renal Physiol. 2010;298:F870-82 pubmed publisher

More Information

Publications62

  1. Hecht N, Omoloja A, Witte D, Canessa L. Evolution of antiglomerular basement membrane glomerulonephritis into membranous glomerulonephritis. Pediatr Nephrol. 2008;23:477-80 pubmed
    ..Even more rare is the evolution of anti-GBM GN into membranous nephropathy (MN). We report the management of a 9-year-old Caucasian girl with anti-GBM GN that evolved into MN and briefly review the literature...
  2. Wu H, Wen Y, Chen M, Fan C. Pulmonary-renal syndrome in a patient with bacterial endocarditis. J Formos Med Assoc. 2005;104:588-92 pubmed
    ..The patient survived and remained on regular hemodialysis. We conclude that bacterial endocarditis should be included in the differential diagnosis of pulmonary-renal syndrome...
  3. Salam N, Rezki H, Fadili W, Hachim K, Ramdani B. Goodpasture's syndrome - four case reports. Saudi J Kidney Dis Transpl. 2007;18:235-8 pubmed
    ..Renal failure was severe and hemodialysis was required in all cases. All patients were treated with prednisone and cyclophosphamide and none recovered renal function. Two patients died due to severe lung hemorrhage...
  4. Ikezumi Y, Hurst L, Masaki T, Atkins R, Nikolic Paterson D. Adoptive transfer studies demonstrate that macrophages can induce proteinuria and mesangial cell proliferation. Kidney Int. 2003;63:83-95 pubmed
    ..Therefore, an adoptive transfer approach was used to directly assess the potential of macrophages to induce renal injury...
  5. van Galen K, Oswald L, van den Aardweg J, Stam F, Van Den Berg J, Bax W. [Variable presentation of antiglomerular basement membrane (anti-GBM) disease: rapid recognition vitally important]. Ned Tijdschr Geneeskd. 2009;153:B365 pubmed
    ..Therefore, despite heterogeneous clinical presentation, immediate recognition of anti-GBM disease is vitally important in view of the potentially lethal consequences if left untreated...
  6. Földes Papp Z, Demel U, Tilz G. A new concept for ultrasensitive fluorescence measurements of molecules in solution and membrane: 2. The individual immune molecule. J Immunol Methods. 2004;286:13-20 pubmed
    ..The novel universal theoretical and experimental framework for continuous measuring the same single, individual immune molecule can be readily transferred to other applications...
  7. Persson U, Hertz J, Carlsson M, Hellmark T, Juncker I, Wieslander J, et al. Patients with Goodpasture's disease have two normal COL4A3 alleles encoding the NC1 domain of the type IV collagen alpha 3 chain. Nephrol Dial Transplant. 2004;19:2030-5 pubmed
  8. Iwatsubo S, Fujimoto S, Matsumoto M, Sato Y, Hara S, Kitamura K, et al. Increased production of adrenomedullin in glomeruli from anti-glomerular basement membrane glomerulonephritis rats treated with methylprednisolone. Nephron Exp Nephrol. 2006;104:e41-7 pubmed
    ..Glucocorticoids are widely used for the treatment of GN; however, the relationship between AM regulation in MCs or glomeruli and glucocorticoid treatment has not been clarified...
  9. Borza D, Bondar O, Colon S, Todd P, Sado Y, Neilson E, et al. Goodpasture autoantibodies unmask cryptic epitopes by selectively dissociating autoantigen complexes lacking structural reinforcement: novel mechanisms for immune privilege and autoimmune pathogenesis. J Biol Chem. 2005;280:27147-54 pubmed
    ..These findings provide fundamental new insights into immune privilege and the molecular mechanisms underlying the pathogenesis of human autoimmune Goodpasture disease...
  10. Le Quang C, Delevaux I, Trouillier S, Andre M, Souweine B, Tridon A, et al. [Intra-alveolar haemorrhage without renal damage as the initial presenting feature of Goodpasture's syndrome: case report and review of literature]. Rev Med Interne. 2008;29:1038-42 pubmed publisher
    ..Goodpasture's syndrome without renal abnormality could be an early stage of the disease with a better prognosis...
  11. Saegusa Y, Sadakane C, Koseki J, Hasegawa Y, Shindo S, Maruyama H, et al. TJN-331 improves anti-glomerular basement membrane nephritis by inhibiting the production of intraglomerular transforming growth factor-beta1. Biol Pharm Bull. 2010;33:1349-54 pubmed
    ..These results suggest that TJN-331 inhibits proteinuria and histopathological changes in glomeruli via suppression of TGF-beta1 production from inflamed glomeruli...
  12. Bernal D, Quinones S, Saus J. The human mRNA encoding the Goodpasture antigen is alternatively spliced. J Biol Chem. 1993;268:12090-4 pubmed
    ..Furthermore, the antigen sequence was identical in the kidneys of normal and Goodpasture-affected individuals, and no major differences in the expression of the complete and spliced forms were observed...
  13. Hudson B. The molecular basis of Goodpasture and Alport syndromes: beacons for the discovery of the collagen IV family. J Am Soc Nephrol. 2004;15:2514-27 pubmed
  14. Kitagawa W, Imai H, Komatsuda A, Maki N, Wakui H, Hiki Y, et al. The HLA-DRB1*1501 allele is prevalent among Japanese patients with anti-glomerular basement membrane antibody-mediated disease. Nephrol Dial Transplant. 2008;23:3126-9 pubmed publisher
    ..We aimed to clarify the relationship between HLA-DRB1(*)1501 and anti-glomerular basement membrane (GBM) antibody-mediated disease in Japanese patients...
  15. Assi M, Berg J, Marshall W, Wengenack N, Patel R. Mycobacterium gordonae pulmonary disease associated with a continuous positive airway pressure device. Transpl Infect Dis. 2007;9:249-52 pubmed
    ..We describe the case of a kidney transplant recipient who developed Mycobacterium gordonae pneumonia transmitted by his continuous positive airway pressure (CPAP) machine...
  16. Picard C, Parrot A, Mayaud C, Cadranel J. [Diffuse alveolar hemorrhage in the immunocompetent host: diagnostic and therapeutic management]. Presse Med. 2009;38:1343-52 pubmed publisher
    ..Whatever is the cause, diffuse alveolar hemorrhage is an emergency associated with an intrahospital mortality rate of approximately 20 percent...
  17. Sheikh Hamad D. Mammalian stanniocalcin-1 activates mitochondrial antioxidant pathways: new paradigms for regulation of macrophages and endothelium. Am J Physiol Renal Physiol. 2010;298:F248-54 pubmed publisher
    ..Our data suggest that STC1 activates antioxidant pathways in endothelial cells and macrophages and displays cytoprotective and anti-inflammatory actions...
  18. Wenderfer S, Dubinsky W, Hernandez Sanabria M, Braun M. Urine proteome analysis in murine nephrotoxic serum nephritis. Am J Nephrol. 2009;30:450-8 pubmed publisher
    ..In mice, large concentrations of albumin and lipocalins have complicated the ability to identify urinary biomarkers in disease models...
  19. Arzoo K, Sadeghi S, Liebman H. Treatment of refractory antibody mediated autoimmune disorders with an anti-CD20 monoclonal antibody (rituximab). Ann Rheum Dis. 2002;61:922-4 pubmed
    ..Rituximab, a chimeric monoclonal anti-CD20 antibody, has recently been used for the treatment of refractory antibody mediated autoimmune diseases such as immune mediated thrombocytopenia and haemolytic anaemia...
  20. Meyers K, Allen J, Gehret J, Jacobovits A, Gallo M, Neilson E, et al. Human antiglomerular basement membrane autoantibody disease in XenoMouse II. Kidney Int. 2002;61:1666-73 pubmed
    ..Availability of engineered mice that produce antibodies (that is, XenoMouse II strains) provides an ideal opportunity to examine the human antibody response...
  21. Shou I, Tomino Y. [Goodpasture syndrome]. Nihon Rinsho. 2002;60 Suppl 1:112-9 pubmed
  22. Nakamura A, Yuasa T, Ujike A, Ono M, Nukiwa T, Ravetch J, et al. Fcgamma receptor IIB-deficient mice develop Goodpasture's syndrome upon immunization with type IV collagen: a novel murine model for autoimmune glomerular basement membrane disease. J Exp Med. 2000;191:899-906 pubmed
    ..These results highlight the role of FcgammaRIIB in maintaining tolerance and suggest that it may play a role in the pathogenesis of human GPS...
  23. Phoon R, Kitching A, Odobasic D, Jones L, Semple T, Holdsworth S. T-bet deficiency attenuates renal injury in experimental crescentic glomerulonephritis. J Am Soc Nephrol. 2008;19:477-85 pubmed publisher
    ..We conclude that T-bet directs Th1 responses that induce renal injury in experimental crescentic glomerulonephritis...
  24. Hamano Y, Okude T, Shirai R, Sato I, Kimura R, Ogawa M, et al. Lack of collagen XVIII/endostatin exacerbates immune-mediated glomerulonephritis. J Am Soc Nephrol. 2010;21:1445-55 pubmed publisher
    ..Taken together, these results suggest that collagen XVIII/endostatin preserves the integrity of the extracellular matrix and capillaries in the kidney, protecting against progressive glomerulonephritis...
  25. Xie C, Rahman Z, Xie S, Zhu J, Du Y, Qin X, et al. Strain distribution pattern of immune nephritis--a follow-up study. Int Immunol. 2008;20:719-28 pubmed publisher
    ..Studies aimed at elucidating the genetic factors and molecular pathways responsible for the enhanced renal disease in C58 mice are warranted...
  26. Bakkaloglu S, Kasapkara C, Soylemezoglu O, Peru H, Fidan K, Hasanoglu E, et al. Successful management of anti-GBM disease in a 5 1/2-year-old girl. Nephrol Dial Transplant. 2006;21:2979-81 pubmed
  27. Upshaw J, Panzarino V, Self S. Anemia, cough, and acute renal failure in a teenager: case presentation and review. Fetal Pediatr Pathol. 2007;26:119-24 pubmed
    ..We present a case where these common symptoms led to the discovery of an uncommon entity, Goodpasture's Syndrome. The purpose of this report is to review the clinical manifestations, treatment and pathology of Goodpasture's Syndrome...
  28. Hirayama K, Yamagata K, Kobayashi M, Koyama A. Anti-glomerular basement membrane antibody disease in Japan: part of the nationwide rapidly progressive glomerulonephritis survey in Japan. Clin Exp Nephrol. 2008;12:339-347 pubmed publisher
    ..9%, and the mortality at 6 months after onset was 23.3%. To improve the prognosis for anti-GBM antibody disease, it may be necessary to detect this disease in the early stages and to treat it without delay...
  29. Gunwar S, Ballester F, Kalluri R, Timoneda J, Chonko A, Edwards S, et al. Glomerular basement membrane. Identification of dimeric subunits of the noncollagenous domain (hexamer) of collagen IV and the Goodpasture antigen. J Biol Chem. 1991;266:15318-24 pubmed
  30. Zhang Y, Su S, Hecox D, Brady G, Mackin K, Clark A, et al. Central tolerance regulates B cells reactive with Goodpasture antigen alpha3(IV)NC1 collagen. J Immunol. 2008;181:6092-100 pubmed
    ..The central tolerance phenotype implies that tolerizing self-Ag is expressed in bone marrow...
  31. Okada H, Inoue T, Kanno Y, Kobayashi T, Ban S, Kalluri R, et al. Renal fibroblast-like cells in Goodpasture syndrome rats. Kidney Int. 2001;60:597-606 pubmed
    ..Interstitial fibroblast-like cells (FbLCs) are presumably involved in the fibrotic process. However, such FbLCs have never been well characterized in the kidney...
  32. Sheerin N, Springall T, Abe K, Sacks S. Protection and injury: the differing roles of complement in the development of glomerular injury. Eur J Immunol. 2001;31:1255-60 pubmed
    ..These data illustrate the potential for the net effect of complement to switch from a detrimental to a protective mode at different stages of autoimmune injury...
  33. Phelps R, Rees A. The HLA complex in Goodpasture's disease: a model for analyzing susceptibility to autoimmunity. Kidney Int. 1999;56:1638-53 pubmed
    ..Towards this goal, we present a detailed analysis of HLA associations with the disease and examine molecular mechanisms that could account for them...
  34. Tipping P, Huang X, Qi M, Van G, Tang W. Crescentic glomerulonephritis in CD4- and CD8-deficient mice. Requirement for CD4 but not CD8 cells. Am J Pathol. 1998;152:1541-8 pubmed
    ..They support the hypothesis that crescent formation is a manifestation of CD4-dependent (and CD8-independent) delayed type hypersensitivity in the glomerulus...
  35. Poddar B, Singhal S, Azim A, Gulati S, Baronia A. Goodpasture's syndrome in children. Saudi J Kidney Dis Transpl. 2010;21:935-9 pubmed
    ..Goodpasture's syndrome is an autoimmune disease that has rarely been described in children, and only 21 cases have been reported till now. Here is a case of a 9-year-old boy who was diagnosed to have Goodpasture's syndrome...
  36. Clatworthy M, Wallin E, Jayne D. Anti-glomerular basement membrane disease after alemtuzumab. N Engl J Med. 2008;359:768-9 pubmed publisher
  37. Ho J, Gibson I, Zacharias J, Fervenza F, Colon S, Borza D. Antigenic heterogeneity of IgA anti-GBM disease: new renal targets of IgA autoantibodies. Am J Kidney Dis. 2008;52:761-5 pubmed publisher
  38. Sydor A, Siudzińska J, Dubiel Bigaj M, Plezia B, Okon K. [Goodpasture syndrome--diagnostic problems]. Przegl Lek. 2004;61:523-7 pubmed
    ..In the course of 6 years' treatment, the patient's condition is described as very good with no recurrence of haemoptysis. On June 30th 2003 renal transplantation was performed...
  39. Hochegger K, Siebenhaar F, Vielhauer V, Heininger D, Mayadas T, Mayer G, et al. Role of mast cells in experimental anti-glomerular basement membrane glomerulonephritis. Eur J Immunol. 2005;35:3074-82 pubmed
    ..Our report provides the first direct evidence that MC are protective in anti-GBM GN, possibly by modulating the influx of effector T cells and macrophages to inflammatory sites in the kidney...
  40. Savige J. Thin basement membrane nephropathy and coincidental renal biopsy lesions. Nephrology (Carlton). 2004;9:52 pubmed
  41. Ohse T, Ota T, Kieran N, Godson C, Yamada K, Tanaka T, et al. Modulation of interferon-induced genes by lipoxin analogue in anti-glomerular basement membrane nephritis. J Am Soc Nephrol. 2004;15:919-27 pubmed
    ..Microarray analysis detected the change of mRNA expression in anti-GBM nephritis and demonstrated amelioration of various genes by ATLa, which may provide a clue to the development of novel therapeutic approaches in immune renal injury...
  42. Wu J, Arends J, Borillo J, Zhou C, Merszei J, McMahon J, et al. A self T cell epitope induces autoantibody response: mechanism for production of antibodies to diverse glomerular basement membrane antigens. J Immunol. 2004;172:4567-74 pubmed
    ..We conclude that autoantibody response to diverse native GBM Ags was induced by a single nephritogenic T cell epitope. Thus, anti-GBM Ab may actually be a consequence of T cell-mediated glomerulonephritis...
  43. Charytan D, Le D, Grossman J, Holthaus K, Kalluri R. Antiglomerular basement membrane autoantibodies are nonpathogenic in Wegener's granulomatosis. Am J Med. 2003;115:414-5 pubmed
  44. Yokoo T. Inflamed site-specific delivery of bone marrow-derived cells carrying IL-1Ra. Nephrol Dial Transplant. 2002;17 Suppl 9:91-3 pubmed
    ..These data suggested that our novel system may be used as a therapeutic intervention for glomerulonephritis...
  45. Zidar N, Volavsek M, Trcek C, Kern I, Gale N. Wegener's granulomatosis in the upper respiratory tract. Wien Klin Wochenschr. 2000;112:676-9 pubmed
    ..We emphasise the significance of clinical, histological and serological parameters in the early detection of Wegener's granulomatosis...
  46. Phelps R, Jones V, Coughlan M, Turner A, Rees A. Presentation of the Goodpasture autoantigen to CD4 T cells is influenced more by processing constraints than by HLA class II peptide binding preferences. J Biol Chem. 1998;273:11440-7 pubmed
  47. Gartenschläger M, Lenci G, Schinzel H, Schweden F, Dienes H, Weilemann L. [Goodpasture syndrome: treatment initiation with plasmapheresis before histologic diagnostic verification]. Pneumologie. 1996;50:764-8 pubmed
    ..Renal biopsy was performed after the acute phase and showed glomerulonephritis and linear immunoglobulin deposition along the GBM. Radiologic findings at CXR and high resolution computed tomography are demonstrated...
  48. Butkowski R, Langeveld J, Wieslander J, Hamilton J, Hudson B. Localization of the Goodpasture epitope to a novel chain of basement membrane collagen. J Biol Chem. 1987;262:7874-7 pubmed
    ..Alternatively, they could represent new molecular species of basement membrane collagen containing a globular domain, comprising M2* and M3, with physicochemical properties very similar to those of collagen IV...
  49. Kashtan C. Autotopes and allotopes. J Am Soc Nephrol. 2005;16:3455-7 pubmed
  50. Kitching A, Turner A, Semple T, Li M, Edgtton K, Wilson G, et al. Experimental autoimmune anti-glomerular basement membrane glomerulonephritis: a protective role for IFN-gamma. J Am Soc Nephrol. 2004;15:1764-74 pubmed
    ..They lend weight to the hypothesis that depending on the context/severity of the nephritogenic immune response IFN-gamma has different effects...
  51. Petit J, Murat O, Perrier S, Lepouse C, Jaussaud M, Leon A. [Headaches in a 21-year-old man with Goodpasture disease]. Ann Fr Anesth Reanim. 2009;28:799-802 pubmed publisher
    ..The case of a 21-year old man who died due to an intracranial thrombosis just after diagnosis of Goodpasture's disease, is reported. Discussion deals with the putative mechanisms, which could be responsible for the thrombosis...
  52. Turner A. Antibodies: still important in anti-GBM disease. Nephron Clin Pract. 2003;94:c51-2 pubmed
  53. Pérez Suárez G, Marrero D, Rodriguez R, Delgado P, Cobo M, Gonzalez Posada J, et al. [Goodpasture's syndrome with neurologic involvement and negative ANCA]. Nefrologia. 2010;30:584-7 pubmed publisher
    ..Twenty months later, with the disease in remission, he underwent deceased-donor renal transplantation...