scholarly journals Goodpasture’s Syndrome and p-ANCA Associated Vasculitis in a Patient of Silicosiderosis: An Unusual Association

2014 ◽  
Vol 2014 ◽  
pp. 1-7
Author(s):  
Amanjit Bal ◽  
Ashim Das ◽  
Dheeraj Gupta ◽  
Mandeep Garg

Introduction.Goodpasture’s syndrome is a rare clinical entity and is characterized by circulating autoantibodies which are principally directed against the glomerular/alveolar basement membrane. The etiology of Goodpasture’s syndrome is still unknown. Lung involvement occurs as a result of lung injury and the exposure of new epitopes to the immune system. Recently, several studies have suggested the role of silica as one of etiological factors in ANCA associated vasculitis and glomerulonephritis.Materials and Methods.We present a case of a 40-year-old welder with silicosiderosis, who developed anti-GBM disease with p-ANCA positivity.Case Report.Patient presented to an emergency with gradually increasing breathlessness along with renal failure and died after short hospital stay. Autopsy pathology findings revealed crescentic glomerulonephritis with linear glomerular basement membrane antibody deposition, splenic vasculitis, pulmonary haemorrhage, and pulmonary silicosiderosis.Conclusion.This case reinforces the role of environmental triggers like exposure to silica, metal dust, and tobacco in pathogenesis of Goodpasture’s syndrome and p-ANCA associated vasculitis.

2012 ◽  
Vol 5 (6) ◽  
pp. 545-548 ◽  
Author(s):  
G. Moulis ◽  
A. Huart ◽  
J. Guitard ◽  
F. Fortenfant ◽  
D. Chauveau

2005 ◽  
Vol 21 (2) ◽  
pp. 526-529 ◽  
Author(s):  
Matthias Alexander Hellmann ◽  
Thomas Michael Gerhardt ◽  
Christian Rabe ◽  
Susanne Haas ◽  
Tilman Sauerbruch ◽  
...  

2000 ◽  
Vol 191 (5) ◽  
pp. 899-906 ◽  
Author(s):  
Akira Nakamura ◽  
Takae Yuasa ◽  
Azusa Ujike ◽  
Masao Ono ◽  
Toshihiro Nukiwa ◽  
...  

The combination of hemorrhagic pneumonitis and rapidly progressive glomerulonephritis is a characteristic feature of Goodpasture's syndrome (GPS), an autoimmune disease resulting from the interaction of pathogenic anti–collagen type IV (C-IV) antibodies with alveolar and glomerular basement membranes. Lack of a suitable animal model for this fatal disease has hampered both a basic understanding of its etiology and the development of therapeutic strategies. We now report a novel model for GPS using mice deficient in a central regulatory receptor for immunoglobulin (Ig)G antibody expression and function, the type IIB Fc receptor for IgG (FcγRIIB). Mutant mice immunized with bovine C-IV reproducibly develop massive pulmonary hemorrhage with neutrophil and macrophage infiltration and crescentic glomerulonephritis. The distinctive linear, ribbon-like deposition of IgG immune complex seen in GPS was observed along the glomerular and tubulointerstitial membranes of diseased animals. These results highlight the role of FcγRIIB in maintaining tolerance and suggest that it may play a role in the pathogenesis of human GPS.


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