scholarly journals Atypical Goodpasture’s disease: a clinical case report and literature review

2018 ◽  
Vol 90 (6) ◽  
pp. 130-136 ◽  
Author(s):  
M L Bulanova ◽  
D V Potapov ◽  
N M Bulanov ◽  
L V Lysenko(Kozlovskaya)
Keyword(s):  
Basement Membrane ◽  
Renal Involvement ◽  
Rapidly Progressive Glomerulonephritis ◽  
Young Male ◽  
Alveolar Hemorrhage ◽  
Disease Course ◽  
Small Vessels ◽  
Goodpasture's Disease ◽  
Pulmonary Renal Syndrome ◽  
Goodpasture’S Disease

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.

Antiglomerular Basement Membrane Disease

2018 ◽  
Vol 39 (04) ◽  
pp. 494-503 ◽  
Author(s):  
Charles Pusey ◽  
Stephen McAdoo
Keyword(s):  
Basement Membrane ◽  
Rapidly Progressive Glomerulonephritis ◽  
Basement Membranes ◽  
Alveolar Hemorrhage ◽  
Type Iv ◽  
Maintenance Immunosuppression ◽  
Life Threatening ◽  
Renal Prognosis ◽  
Function Testing

AbstractAntiglomerular basement membrane (anti-GBM) disease is a rare but life-threatening autoimmune vasculitis that is characterized by the development of pathogenic autoantibodies to type IV collagen antigens expressed in the glomerular and alveolar basement membranes. Once deposited in tissue, these autoantibodies incite a local capillaritis which manifests as rapidly progressive glomerulonephritis (GN) in 80 to 90% of patients, and with concurrent alveolar hemorrhage in ∼50%. A small proportion of cases may present with pulmonary disease in isolation. Serological testing for anti-GBM antibodies may facilitate rapid diagnosis, though renal biopsy is often required to confirm the presence of necrotizing or crescentic GN and linear deposition of autoantibody on the glomerular basement membrane. Alveolar hemorrhage may be evident clinically, or detected on imaging, pulmonary function testing, or bronchoscopy. Prompt treatment with plasmapheresis, cyclophosphamide, and steroids is usually indicated to remove pathogenic autoantibodies, to prevent their ongoing production, and to ameliorate end-organ inflammation. Alveolar hemorrhage is usually responsive to this treatment, and long-term respiratory sequelae are uncommon. Renal prognosis is more variable, though with aggressive treatment, independent renal function is maintained at 1 year in more than 80% of patients not requiring renal replacement therapy at presentation. Relapse in uncommon in anti-GBM disease, unless there is a concomitant antineutrophil cytoplasm antibody (present in 30–40%), in which case maintenance immunosuppression is recommended.

Antiglomerular basement membrane disease

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....

scholarly journals An uncommon cause of diffuse alveolar hemorrhage in a young male

2019 ◽  
Vol 89 (2) ◽  
Author(s):  
Anshul Mittal ◽  
Jagdish Chander Suri ◽  
Shibdas Chakrabarti ◽  
Pranav Ish
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Young Male ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Systemic Lupus ◽  
Threatening Condition ◽  
Life Threatening

It is uncommon for Systemic lupus erythematosus (SLE) to present with diffuse alveolar hemorrhage (DAH) as the initial presentation. To diagnose this in a young male with no renal involvement is further uncommon. We report a case of a 16-year-old boy, who presented with hemoptysis and was eventually diagnosed as DAH with underlying SLE. Treatment with steroids and immunosuppressant helped in rapid recovery from this potentially life-threatening condition. This case highlights the need of defining diagnostic criteria for SLE in patients presenting as DAH and formulating guidelines for treatment of the same, especially in absence of co-existing lupus nephritis.

Goodpasture's disease in the absence of circulating anti-glomerular basement membrane antibodies as detected by standard techniques

2002 ◽  
Vol 39 (6) ◽  
pp. 1162-1167 ◽  
Author(s):  
Alan D. Salama ◽  
Tammy Dougan ◽  
Jeremy B. Levy ◽  
H.Terry Cook ◽  
Steve H. Morgan ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Goodpasture's Disease ◽  
Goodpasture’S Disease ◽  
Standard Techniques

Goodpasture's disease: Antiglomerular basement membrane disease

Nephrology ◽  
2004 ◽  
Vol 9 (2) ◽  
pp. 49-51 ◽  
Author(s):  
JONATHAN H ERLICH ◽  
JACOB SEVASTOS ◽  
BRUCE A PUSSELL
Keyword(s):  
Basement Membrane ◽  
Goodpasture's Disease ◽  
Goodpasture’S Disease
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