Handbook of Pediatric Autopsy Pathology by Enid Gilbert-Barness Diane E. Spicer & Thora S. Steffensen
Author:Enid Gilbert-Barness, Diane E. Spicer & Thora S. Steffensen
Language: eng
Format: epub
Publisher: Springer New York, New York, NY
Lupus Nephritis
Twenty-five percent of all cases of SLE present in childhood or adolescence. Arthritis, arthralgia, rash, and fever are the most common presenting complaints, but renal, cardiac, and central nervous system involvement becomes evident as the disease progresses, and urinary or renal function abnormalities develop in 60–80 % of children. Most patients are girls. Renal involvement in SLE is characterized by hematuria, proteinuria, and hypertension.
The 2004 International Society of Nephrology/Renal Pathology Society Classifications are as follows: Class I—minimal mesangial lupus nephritis (LN), where glomeruli are normal by light microscopy (LM) but have mesangial immune deposits by immunofluorescence (IF); Class II—mesangial proliferative LN, where glomeruli show mesangial hypercellularity or matrix expansion without histologic alterations of capillaries; Class III—focal LN; and Class IV—diffuse LN, showing focal (<50 % of glomeruli) or diffuse glomerulonephritis with subendothelial immune deposits. Class V is membranous LN and is diagnosed, alone or in combination, with Class III or IV, when there are subepithelial immune deposits or their sequelae over greater than 50 % of the capillary wall; class VI indicates global sclerosis of 90 % or more of glomeruli without evidence of activity. Chronic disease includes glomerulosclerosis, fibrous crescents, tubular atrophy, and interstitial fibrosis.
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