Two cases of anti-glomerular basement membrane (Goodpasture’s) disease in female adolescents

Goodpasture’s disease (anti-GBM disease) is a rare small vessels vasculitis characterized by the presence of autoantibodies directed against the glomerular basement membrane (GBM) and alveolar basement membrane. Common feature of anti-GBM disease is a combination of rapidly progressive glomerulonephritis and alveolar hemorrhage (pulmonary-renal syndrome). We present a case of atypical disease course in a young male patient who developed alveolar hemorrhage without renal failure. The only symptom of renal involvement was isolated hematuria. Plasmapheresis combined with immunosuppression (cyclophosphamide and corticosteroids) was effective. We present a review of state-of-art data on the pathogenesis and disease course of anti-GBM disease.

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Antiglomerular Basement Membrane Disease and Goodpasture's Disease

Comprehensive Clinical Nephrology ◽

10.1016/b978-0-323-05876-6.00023-x ◽

2010 ◽

pp. 282-291 ◽

Cited By ~ 3

Author(s):

Richard G. Phelps ◽

A. Neil Turner

Keyword(s):

Basement Membrane ◽

Goodpasture's Disease ◽

Goodpasture’S Disease

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Antiglomerular basement membrane disease

Oxford Textbook of Medicine ◽

10.1093/med/9780199204854.003.210807_update_002 ◽

2010 ◽

pp. 3995-4001

Author(s):

Jeremy Levy ◽

Charles Pusey

Keyword(s):

Autoimmune Disease ◽

Basement Membrane ◽

Type Iv Collagen ◽

Complement C3 ◽

Terminal Domain ◽

Type Iv ◽

Goodpasture's Disease ◽

Goodpasture’S Disease

Antiglomerular basement membrane disease (anti-GBM disease, also known as Goodpasture’s disease) is a rare autoimmune disease caused by pathogenic autoantibodies directed against the noncollagenous, C-terminal domain of the α‎-3 chain of type IV collagen (α‎3(IV)NC1). Immunohistology is characteristic, with linear deposition of IgG (sometimes with IgA or IgM) and complement C3 along the GBMs....

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Goodpasture's disease: Antiglomerular basement membrane disease

Nephrology ◽

10.1111/j.1440-1797.2004.00244.x ◽

2004 ◽

Vol 9 (2) ◽

pp. 49-51 ◽

Cited By ~ 4

Author(s):

JONATHAN H ERLICH ◽

JACOB SEVASTOS ◽

BRUCE A PUSSELL

Keyword(s):

Basement Membrane ◽

Goodpasture's Disease ◽

Goodpasture’S Disease

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Electron microscopic radioautographic studies on the incorporation of 3H proline in the glomerular basement membrane (GBM) in antistreptococcal-cell-membrane (SCM) injected mice

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100111057 ◽

1984 ◽

Vol 42 ◽

pp. 188-189

Author(s):

R.P. Nayyar ◽

C.F. Lange ◽

J. L. Borke

Keyword(s):

Body Weight ◽

Cell Membrane ◽

Basement Membrane ◽

Glomerular Basement Membrane ◽

Mesangial Matrix ◽

Electron Microscopic ◽

Group A ◽

Injection Protocol

Streptococcal cell membrane (SCM) antiserum injected mice show a significant thickening of glomerular basement membrane (GBM) and an increase in mesangial matrix within 4 to 24 hours of antiserum administration (1,2,3). This study was undertaken to evaluate the incorporation of 3H proline into glomerular cells and GBM under normal and anti-SCM induced conditions. Mice were administered, intraperitoneally, 0.1 ml of normal or anti-SCM serum followed by a 10 µC/g body weight injection of 3H proline. Details of the preparation of anti-SCM (Group A type 12 streptococcal pyogenes) and other sera and injection protocol have been described elsewhere (2). After 15 minutes of isotope injection a chase of cold proline was given and animal sacrificed at 20 minutes, 1,2,4,8,24 and 48 hours. One of the removed kidneys was processed for immunofluorescence, light and electron microscopic radioautographic studies; second kidney was used for GBM isolation and aminoacid analysis.

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