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Lupus Nephritis
Jane Cross
David Jayne
Pathology
Immune deposits in the glomeruli and mesangium are characteristic of SLE, and stain positive for IgG, IgM, IgA and the complement components C3, C1q and C4 on immunofluorescence. Circulating autoantibodies to cellular antigens (particularly anti-dsDNA,
anti-Ro and anti-C1q) and complement activation with correspondingly reduced serum C3, C4 and C1q levels are typical of lupus
nephritis. Following the appearance of immune complexes, an
inflammatory reaction develops with mesangial cell proliferation,
expansion of the mesangial matrix and infiltration of inflammatory
leucocytes. Other pathogenic mechanisms include infarction of
glomerular segments, thrombotic microangiopathy, vasculitis and
Management
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Class II
1.0
Class I
Light microscopy normal, immune deposits on immunofluorescence
Class V
0.8
Class II
A mesangial deposits
B mesangial hypercellularity
Class III
Class I
Survival
0.6
Class III
Focal segmental proliferative glomerulonephritis (< 50% glomeruli)
with leucocyte infiltration; immune deposits in the mesangium and
subendothelium
Class IV
0.4
Class IV
As class III, but diffuse (> 50% glomeruli); may also see glomerular
crescents and fibrinoid necrosis; includes membranoproliferative
variant
0.2
Class VI
Class V
Membranous nephropathy with uniform thickening of the capillary
walls and subepithelial immune complex deposition
A membranous nephropathy alone
B membranous nephropathy + class II
C membranous nephropathy + class III
D membranous nephropathy + class IV
10
15
20
Class VI
Glomerulosclerosis
2
1
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Prognosis
Development of renal disease is the strongest predictor of ESRF and
early mortality in SLE. Renal histology and renal function are the
most important renal prognostic factors at presentation (Figure 2).
Following treatment, normalization of proteinuria and the absence
of relapse of nephritis are the best predictors of a good outcome.
Negative prognostic factors include male gender, black race and
haematological features of SLE.
102
is also associated with fetal loss and premature delivery. Management by a specialist team before conception and during pregnancy
is important in optimizing fetal and renal outcome.
u
Drugs and
Renal Insufficiency
REFERENCES
Balow J E, Boumpas D T, Fessler B J et al. Management of Lupus Nephritis.
Kidney Int 1996; 49: (Suppl. 53): 8892.
Bansal V K, Beto J A. Treatment of Lupus Nephritis: A Meta-analysis of
Clinical Trials. Am J Kidney Dis 1997; 29(2): 1939.
Hochberg M C. Updating the American College of Rheumatology Revised
Criteria for the Classification of Systemic Lupus Erythematosus.
Arthritis Rheum 1997; 40(9): 1725.
Korbet S M, Lewis E J, Schwartz M M et al. Factors Predictive of
Outcome in Severe Lupus Nephritis. Am J Kidney Dis 2000; 35(5):
90414.
Jeffrey K Aronson
FURTHER READING
Davison A M, Cameron J S, Grnfeld J-P et al., eds. Oxford Textbook of
Clinical Nephrology. Vol. 2. 2nd ed. Oxford: Oxford University Press,
1998.
(Includes an excellent review of all aspects of lupus nephritis.)
Balow J E, Austin H A. Progress in the Treatment of Proliferative
Lupus Nephritis. Curr Opin Nephrol Hypertens 2000; 9(2):
10715.
(Reviews potential newer therapies for lupus nephritis.)
Practice points
Lupus nephritis is common in patients with SLE and may be
asymptomatic
Clinical features do not predict severity on renal biopsy
Development of lupus nephritis strongly predicts renal and
patient survival
Current treatment is with cortciosteroids and an
immunosuppressive agent such as intravenous
cyclophosphamide or azathioprine
Large trials are required to confirm optimum therapy and assess
newer, less toxic treatments
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