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Currently filtering by tag: Lupus nephritis

Membranous Lupus Nephritis

The patient is a 19-year-old female who presents with hematuria, nephrotic range proteinuria, and a creatinine of 1.2 mg/dL. She has a recent diagnosis of systemic lupus erythematosus. Figure 1 shows a normal glomerulus without "spikes" and "holes" of the glomerular basement membranes. Figure 2 shows no significant interstitial fibrosis. Figures 3, 4, 5, & 6 shows IgG, kappa, lambda, and C1q respectively. Image 7 and 8 shows subepithelial deposits. This is a case of membranous lupus nephritis (ISN/RPS Class V). By definition, no proliferative changes (crescents, fibrinoid necrosis, endocapillary hypercellularity) are seen within the glomeruli in a pure class...

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. ...

Art of Medicine: Lupus Nephritis Genetics

The above painting shows an image of a sequencing gel, which are produced by DNA synthesis in the presence of sequence terminators such as dideoxyribonucleotides (that are mixed with deoxyribonucleotides used for chain elongation), with the DNA fragments run on an agarose gel. The agarose gels are then imaged using a DNA intercalator, such as ethidium bromide, that allows the DNA fragments to fluoresce in the ultraviolet spectrum. This is the basis of Sanger sequencing. Advances to Sanger sequencing included use of fluorescent labeled chain terminators, which allowed for a higher throughput readout and faster results. These could also be...

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...

Twitter Poll (February 27, 2019)

Answer: B "Endocapillary hypercellularity" is the new term recommended to be used instead of "endocapillary proliferation". This is a more accurate term as an increase in cellularity can be the result of inflammatory influx and does not necessarily requires proliferation. Reference: Bajema IM, et al. Revision of the International Society of Nephrology/Renal Pathology Society classification for lupus nephritis: clarification of definitions, and modified National Institutes of Health activity and chronicity indices. Kidney Int. 2018;93:789-796.    

Lupus Nephritis (Class IV)

A 20-year-old female presents with hematuria, proteinuria, and a creatinine of 1.2 mg/dl. Serologies for ANA are positive. Complement levels are decreased. She states that auto-immune disease runs in her family, but is unsure about any specific diagnosis. Serologies for dsDNA, SSA, SSB, rheumatoid factor, hepatitis B, hepatitis C, and HIV are pending. Figure 1 shows mesangial and endocapillary hypercellularity with prominent "hyaline thrombi." Figure 2 shows no significant interstitial fibrosis. Figure 3 shows "wire loops" and no "spikes" and "holes." Figure 4, Figure 5, and Figure 6 shows mesangial and capillary staining with IgG, kappa, and lambda, respectively. Figure...

Immune Deposits in SLE

The ISN/RPS 2004 classification of lupus nephritis is entirely based on glomerular changes. Depending on the presence or absence of active and/or chronic glomerular lesions, cases are allocated into one (and occasionally two) of six possible classes. The most commonly encountered active glomerular lesions in clinical practice are in the form of endocapillary or extracapillary proliferation, fibrinoid necrosis or cellular crescents. However, deposition of large subendothelial immune deposits, even in the absence additional proliferative/necrotizing lesions is sufficient to classify a case as class III or IV, depending on the number of involved glomeruli (Fig 1-3). These deposits, termed “wire loops”,...