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Disease Week: Lupus Nephritis

Monday:  Introduction to Systemic Lupus Erythematosus and Clinical Diagnostic Criteria Answer:  C.  50%  50% of adults with SLE will develop lupus nephritis.   In children with SLE, 80% have nephritis. References: Madhok R.  Systemic lupus erythematosus: lupus nephritis.  BMJ Clinical Evidence 2015 Dec 18; pp 1123. https://www.ncbi.nlm.nih.gov/pubmed/26683208 Liu CC, Kao AH, Manzi S, Ahearn JM.  Biomarkers in systemic lupus erythematosus: challenges and prospects for the future.  Therapeutic Advances in Musculoskeletal Disease 2013; 5 (4): 210-233. https://www.ncbi.nlm.nih.gov/pubmed/23904865 In a majority of cases, renal involvement in SLE often occurs in the first year following diagnosis, but can occur at any time in the disease course. ...

NIH Lupus Nephritis Activity and Chronicity Indices

NIH Lupus Nephritis Activity and Chronicity Indices: A way to monitor response to treatment and disease progression. A semi-quantitative grading system of pathologic features on kidney biopsies allows for monitoring response to treatment and showing disease progression. Lupus nephritis disease activity can be assessed on a renal biopsy using the modified NIH activity and chronicity indices. Indicators of disease activity include endocapillary hypercellularity, neutrophils or karyorrhexis within glomerular capillary loops, fibrinoid necrosis, hyaline deposits, cellular or fibrocellular crescents, and interstitial inflammation. Crescents and fibrinoid necrosis are weighted twice as they have a worse impact on prognosis. The scoring is based...

International Society of Nephrology / Renal Pathology Society Classification of Lupus Nephritis

How do we classify lupus nephritis on a renal biopsy?  SLE patients with an active urinary sediment or proteinuria may undergo a kidney biopsy to evaluate for nephritis.  The International Society of Nephrology (ISN) and Renal Pathology Society (RPS) created a lupus nephritis classification system based on morphologic findings on kidney biopsies.  A simplified view of the classification algorithm is based on the sites of immune deposits within glomeruli.  A flow-chart demonstrating that algorithm is shown below.   Mesangial immune deposits can be present in all classes. A summary in tabular format is shown below.   While a majority of SLE patients...

Introduction to Systemic Lupus Erythematosus and Clinical Diagnostic Criteria

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease which affects over 5 million people worldwide, and manifests as tissue damage within multiple organ systems.  It has a complex etiology, involving a combination of multiple genetic and environmental factors.  Women are disproportionately affected, with a 9:1 female-to-male ratio. The diagnostic criteria are determined by the American College of Rheumatology (ACR) and the European League against Rheumatism (EULAR).  In the 1997 revision of the 1982 ACR diagnostic criteria for diagnosis of SLE, 4 of 11 disease manifestations are required (Hochberg, 1997).  These are shown below: Notice that “renal disorder” is one...