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Archive: Disease Week

Disease Week: Infection-Associated Glomerulonephritis and Endocarditis-Associated Glomerulonephritis

A paradigm shift has occurred in the epidemiology, bacteriology, and clinical and pathologic findings of infection-associated glomerulonephritis over the past few decades.  The incidence of acute Streptococcal glomerulonephritis has declined, in part due to improved socioeconomic conditions and the advent of antibiotic therapy in developed countries.  An increase in methicillin-resistant and sensitive Staphylococcal infections ranging from superficial skin infections to deep-seated occult infections such as visceral abscess, osteomyelitis, and endocarditis has been noted.  This increase is seen in the expanding elderly population who have increasing comorbidities of diabetes and heart disease and increased use of cardiac devices, indwelling catheters and...

Disease Week: Onco-Nephrology

The rapidly evolving field of oncology and cancer management creates unique opportunities as well as challenges for the nephrology community.  As such, it is important for nephrologists and nephropathologists to be informed and involved in its many aspects in order to better care for patients and be valuable members of a multidisciplinary cancer care team.  The renal manifestations and complications of cancer are myriad and include acute as well as chronic kidney injury, electrolyte/acid–base disturbances, direct injury of the kidney through tumor infiltration and/or paraneoplastic substances, pharmacokinetics of chemotherapeutic agents, and hematopoietic cell transplantation-related kidney injury.  Kidney transplant patients also...

Disease Week: Monoclonal Gammopathy of Renal Significance

Disease Week: Monoclonal Gammopathy of Renal Significance   Summary: Renal disease caused by parenchymal deposition of circulating monoclonal immunoglobulins (mIg) is a well established phenomenon. Light chain cast nephropathy, light chain deposition disease and AL amyloidosis are probably the best known entities; nevertheless, the list also includes a number of less common and recently described entities, some of which are still poorly understood.  These disorders may be seen in the setting of either a B-cell or plasma cell proliferative disorder with production of a mIg. When these proliferative disorders represent an overtly malignant disease (e.g. multiple myeloma, Waldenström macroglobulinemia, high-grade...

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...

Disease Week: C3 Glomerulonephritis

C3 glomerulonephritis is a recently described entity which is due to dysregulation in the alternative complement pathway. Patients typically present with hematuria and/or proteinuria in the face of persistently low serum levels of C3. The annual incidence of biopsy-proven disease is 1 to 2 per million with both sexes affected equally. The median age of diagnosis is 21 years of age, but there is a second spike after the age of 50 due to paraprotein-associated disease. The most common glomerular disease pattern is a membranoproliferative pattern. The hallmark of the disease is dominant C3 staining on immunofluorescence which is defined...

Disease Week: Renal Manifestations of Inflammatory Bowel Disease (IBD)

Inflammatory bowel disease (IBD) is a condition characterized by chronic inflammation of the gastrointestinal tract with the two most common types Crohn disease and ulcerative colitis.  While renal and urinary involvement in IBD is not uncommon, renal parenchymal disease is rare and most commonly affects the glomerular and tubulointerstitial compartments. The most common findings on renal biopsy of IBD patients are IgA nephropathy and tubulointerstitial nephritis, and this occurrence may represent a common pathogenic mechanism. Although several cases of tubulointerstitial nephritis have been related to drug exposure, there is increasing evidence that this finding may also represent a true extraintestinal...

Disase Week: Collagen Type III Glomerulopathy

Collagen type III glomerulopathy is an ultra-rare kidney disease of unknown etiology and pathogenesis with only rare cases reported in the literature, the vast majority being case reports. A few non-controlled, small case series exist that shed light on differences in pediatric and adult presentations and specific geographic patient populations. Type III collagen is typically absent in normal glomeruli, although small amounts have been reported in some advanced glomerular diseases. In patients with collagen type III glomerulopathy, abundant type III collagen is seen within the mesangium and lamina rara interna of the capillary loops and typically occurs in a flocculent...