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Lupus Nephritis

Lupus Nephritis. Emily Chang April 13, 2010. The “Glom”. Classification. Based exclusively on glomerular pathology WHO classification last revised in 2004 Definitions Diffuse: involving >50% glomeruli Focal: involving <50% glomeruli

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Lupus Nephritis

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  1. Lupus Nephritis • Emily Chang • April 13, 2010

  2. The “Glom”

  3. Classification • Based exclusively on glomerular pathology • WHO classification last revised in 2004 • Definitions • Diffuse: involving >50% glomeruli • Focal: involving <50% glomeruli • Global: involving more than half of the glomerular tuft • Segmental: involving less than half of the glomerular tuft

  4. Other forms of Lupus Renal Disease • Tubulointerstitial nephritis: Severity of involvement is an important prognosticator. Almost always seen with concurrent glomerular disease. Sometimes it is the only manifestation of lupus nephritis. • Vascular disease: Presence can adversely affect prognosis of renal disease. Most frequently seen is immune complex deposition leading to vascular narrowing, immunoglobulin microvascular casts, TMA and vasculitis. • Drug-induced lupus: Hydralazine, procainamide, isoniazid.

  5. MKSAP • A 29 yo black woman comes for a routine exam. She has a 3-yr h/o SLE. She recently was diagnosed with a progressive kidney disease characterized by glomerular hematuria with dysmorphic erythrocytes and proteinuria. Over the past 3 weeks, her Cr has risen from 0.9 to 1.4. • Lab studies: C3 60, C4 8, ANA +, anti-ds-DNA + • Kidney biopsy reveals diffuse proliferative immune complex-laden glomerulonephritis with endothelial tubular reticular inclusion bodies.

  6. MKSAP, cont’d. • In addition to corticosteroid therapy, which of the following is the most appropriate treatment for this patient? • A. IV cyclophosphamide followed by maintenance IV cyclophosphamide. • B. IV cyclophosphamide followed by maintenance MMF. • C. Oral cyclophosphamide. • D. IV cyclophosphamide.

  7. References • Weening, Jan J. et al. The classification of glomerulonephritis in systemic lupus erythematosus revisited. Kidney International, Vol. 65 (2004), pp. 521-530. • Cotran, Ramzi S., Kumar, Vinay, Robbins, Stanley L. Robbins Basic Pathology, 7th edition. Saunders. 2003. • MKSAP 14. Nephrology. • utdol.com. Types of renal disease in systemic lupus erythematosus.

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