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Anti-glomerular Basement Disease: Goodpasture’s Syndrome

  • Gangadhar Taduri
  • Raghu Kalluri
  • Ralph J. Panos
Chapter
Part of the Respiratory Medicine book series (RM)

Abstract

Goodpasture’s syndrome is an exemplary rare lung and kidney disease that has led to significant discoveries in human biology. The initial observations that antibodies directed against glomerular basement membrane (GBM) caused glomerulonephritis stimulated evaluation of the components of the basement membrane, elucidation of the reticular collagen network, and identification of different types of collagen. The α3[IV] NC1 domain of type IV collagen is the antigenic epitope that initiates a complex autoimmune reaction culminating in the clinical manifestations of Goodpasture’s syndrome. Immunization with α3[IV] collagen has provided an experimental model that has led to fundamental discoveries into the genetic and immune processes precipitating and modulating autoimmune diseases. Initial evaluation of the autoimmune process focused on humoral mechanisms, but more recent studies suggest that the cellular immune process is activated and plays a key role in the pathogenesis of Goodpasture’s syndrome. Hemoptysis occurs in nearly all patients and renal histopathology demonstrates crescentic glomerulonephritis in the majority of cases. Immunofluorescence microscopy reveals the pathognomonic finding of linear deposition of IgG along with the glomerular capillaries and in the lung parenchyma. The diagnosis of anti-GBM disease is confirmed by the presence of circulating antibodies against basement membrane antigen in the correct clinical setting. Based on the pathogenetic mechanisms, therapeutic modalities include both induction and maintenance regimens: induction or initial therapy removes the pathogenic anti-GBM antibody by plasmapheresis and maintenance therapy reduces antibody production by immunosuppression. The prognosis of patients with anti-GBM disease depends on the level of renal dysfunction at presentation.

Keywords

Goodpasture’s syndrome anti-glomerular basement membrane disease type IV collagen pulmonary–renal syndrome autoimmune disorder 

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Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Gangadhar Taduri
    • 1
  • Raghu Kalluri
    • 2
  • Ralph J. Panos
    • 3
  1. 1.Department of NephrologyNizam’s Institute of Medical SciencesHyderabadIndia
  2. 2.Department of Medicine, and Biological Chemistry and Molecular PharmacologyCenter for Matrix Biology, Beth Israel DeaconessBostonUSA
  3. 3.Department of Internal MedicineUniversity of Cincinnati School of Medicine and Cincinnati VA Medical CenterCincinnatiUSA

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