This is a variety of proliferative glomerulonephritis in which there is a circulating antibody directed against antigens of the glomerular capillary basement membrane.
Aetiology
The pathogenesis is unknown but in some patients the onset appears to follow viral infection of the respiratory tract. The capillary wall is severely disrupted and fibrinogen passes through to the urinary space to stimulate formation of epithelial crescents. The result is usually a crescentic glomerulonephritis of a type known clinically as Good pasture's syndrome.
Clinical features
Clinically it usually presents as acute renal failure and occurs most commonly in young adult males and in spring. There is cross-reactivity between glomerular basement membrane and pulmonary basement membrane; some patients therefore have associated intrapulmonary haemorrhage and may present with haemoptysis. In such patients the chest X-ray reveals pulmonary infiltration and there may be significant impairment of lung function. In the majority of patients irreversible renal failure develops rapidly.
Pathology
On biopsy the appearances are those of crescentic glomerulonephritis and immunofluorescence microscopy reveals a linear deposition of IgG along glomerular capillary walls.
Management
The diagnosis should be established as early as possible since plasma exchange combined with immunosuppression is higly effective in patients who still retain renal function. There is no general agreement but most would suggest pulse methylprednisolone followed by high- dose corticosteroids (prednisolone 60mg/d) for 4-6 weeks with a subsequent cautious and gradual reduction in dose. After oliguria develops, plasma exchange is ineffective and should be undertaken only for life-threatening intra pulmonary haemorrhage.
Prognosis
The prognosis for renal function is poor; when oliguria develops there is not likely to be any recovery of renal function even with intensive therapy. Intrapulmonary haemorrhage carries a poor prognosis for survival and may occur in these patients in association with any intercurrent febrile illness.
Aetiology
The pathogenesis is unknown but in some patients the onset appears to follow viral infection of the respiratory tract. The capillary wall is severely disrupted and fibrinogen passes through to the urinary space to stimulate formation of epithelial crescents. The result is usually a crescentic glomerulonephritis of a type known clinically as Good pasture's syndrome.
Clinical features
Clinically it usually presents as acute renal failure and occurs most commonly in young adult males and in spring. There is cross-reactivity between glomerular basement membrane and pulmonary basement membrane; some patients therefore have associated intrapulmonary haemorrhage and may present with haemoptysis. In such patients the chest X-ray reveals pulmonary infiltration and there may be significant impairment of lung function. In the majority of patients irreversible renal failure develops rapidly.
Pathology
On biopsy the appearances are those of crescentic glomerulonephritis and immunofluorescence microscopy reveals a linear deposition of IgG along glomerular capillary walls.
Management
The diagnosis should be established as early as possible since plasma exchange combined with immunosuppression is higly effective in patients who still retain renal function. There is no general agreement but most would suggest pulse methylprednisolone followed by high- dose corticosteroids (prednisolone 60mg/d) for 4-6 weeks with a subsequent cautious and gradual reduction in dose. After oliguria develops, plasma exchange is ineffective and should be undertaken only for life-threatening intra pulmonary haemorrhage.
Prognosis
The prognosis for renal function is poor; when oliguria develops there is not likely to be any recovery of renal function even with intensive therapy. Intrapulmonary haemorrhage carries a poor prognosis for survival and may occur in these patients in association with any intercurrent febrile illness.
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