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Crescentic nephritis at Groote Schuur Hospital, South Africa--not a benign disease
R Zent1, R Van Zyl Smit, M Duffield
1Renal Unit, Groote Schuur Hospital, Cape Town, South Africa.
Insights
Post-infectious glomerulonephritis is the most common cause of crescentic nephritis in the developing world, often leading to poor outcomes. Early treatment may improve renal recovery in some patients.
Area of Science:
- Nephrology
- Pathology
- Immunology
Background:
- Crescentic nephritis (CN) etiology varies globally.
- Data on CN in the developing world is limited.
Purpose of the Study:
- To describe the largest series of CN in the developing world.
- To identify common causes and outcomes of CN in this region.
Main Methods:
- Retrospective review of 73 renal biopsies from Groote Schuur Hospital, Cape Town (1977-1991).
- Inclusion criteria: >= 6 glomeruli with >50% crescent formation.
Main Results:
- Post-infectious glomerulonephritis (PIGN) was the most common cause (n=21).
- Systemic lupus erythematosus (SLE) was associated with CN in 15 patients.
- 53% of patients progressed to end-stage renal failure (ESRF); only 12% recovered renal function.
- PIGN had a poor prognosis (38% ESRF).
- Combination therapy (steroids and cyclophosphamide) showed potential benefit in PIGN.
- SLE-associated CN indicated a poor prognosis (87% ESRF or death).
Conclusions:
- PIGN is the leading cause of CN in this developing world setting.
- CN associated with SLE carries a high risk of progression to ESRF or mortality.
- Therapeutic strategies may influence outcomes in PIGN-associated CN.
Abstract:
The etiology of crescentic nephritis (CN) in the developing world differs from that of Europe and North America. This retrospective study of 73 patients is the largest series of CN in the developing world. The records of all renal biopsies performed at Groote Schuur Hospital, Cape Town, South Africa, over thirteen years, between January 1977 and April 1991, were reviewed. Specimens selected for this study had six or more glomeruli and over 50% of these glomeruli had crescent formation. It confirms that post infectious glomerulonephritis (PIGN) (n = 21) is the commonest cause of CN in this setting. In addition there were 15 patients with CN associated with systemic lupus erythematosus (SLE). These two groups make this study unique as they are the largest series of each described in the literature. Thirty-nine (53%) patients in this series progressed to end-stage renal failure (ESRF) and only nine (12%) patients recovered renal function to a normal serum creatinine. Eight (38%) patients in the PIGN group developed ESRF, indicating the poor prognosis of this condition. Six of eight patients in the PIGN group treated with steroids and cyclophosphamide recovered to a serum creatinine level less than 200 mumol/l and only one progressed to ESRF, which may indicate that this form of therapy is beneficial. Thirteen (87%) patients with SLE either developed ESRF or died which suggests that the presence of crescents in this condition is associated with a poor prognosis.