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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...

Light Chain Cast Nephropathy

A 75-year-old female is found to have a creatinine of 4.0 mg/dL on a routine laboratory examination. At the hospital, a CT scan shows lytic lesions within the skull. A SPEP shows an IgG kappa monoclonal protein. A bone marrow biopsy shows 5% plasma cells. A kidney biopsy is requested to look for monoclonal gammopathy of renal significance. Figure 1 shows a normal glomerulus. Figure 2 shows severe interstitial fibrosis. Figures 3 & 4 show abnormal, PAS negative "stiffened" casts with cellular reaction and fracture. Figures 5 & 6 show kappa and lambda immunofluorescence, respectively. This is a case of...

Art of Medicine: Polyomavirus Nephritis

The painting above shows acute tubular injury, reactive changes in tubular epithelial cells, tubulitis, inclusions within tubular epithelium, and interstitial inflammation.  These are morphologic changes that can be seen in polyomavirus nephritis.  BK virus is the most common etiology of polyomavirus nephritis, while JC virus and simian virus 40 (SV40) are less common etiologies.  All are DNA viruses that are non-enveloped and show tropism for the genitourinary tract, especially urothelium (Lusco et al, 2016).  Polyomavirus nephritis is a serious complication affecting approximately 5 to 6 percent of kidney transplants, often as a result of over-immunosuppression.  The prevalence is higher in...

Twitter Poll (May 22, 2019)

ANSWER: C By the Oxford Classification of IgA nephropathy, the findings present are classified as "M1 E1 S1 T1 C0" (where M=Mesangial hypercellularity; E=Endocapillary proliferation; S=Segmental sclerosis; T=Tubular atrophy & interstitial fibrosis; C=Cellular/fibrocellular crescents). REFERENCE: Trimarchi H, et al. Oxford Classification of IgA Nephropathy 2016: an update from the IgA Nephropathy Classification Working Group. Kidney Int 2017; 91(5):1014-21.    

NSH Brochure

It’s Jon! We spotted a photo of one of our histotechs while browsing this year’s NSH - National Society for Histotechnology brochure. We asked for an autograph, but he was too busy processing kidney tissue this morning.     

APOL1: Why Is It Important?

I have been working at Arkana Laboratories (formerly Nephropath) for close to seven years as the Molecular Diagnostics Laboratory Supervisor. One of my first projects here was to isolate DNA from formalin-fixed, paraffin-embedded tissue and get an assay set up to determine variants in the APOL1 gene. At that time, these variants were recently discovered and there was great excitement in the scientific community as to their role in kidney disease, especially in African Americans, which is the population with the highest frequency of these gene variants in America. Our project involved the role of these variants in collapsing glomerulopathy...

Art of Medicine: Minimal Change Disease

The above painting shows podocytes with foot processes extending along the glomerular basement membrane of neighboring capillary loops.  Effacement of podocyte foot processes occurs in primary podocytopathies, including minimal change disease (see electron photomicrograph below). Minimal change disease is the most common etiology of idiopathic nephrotic syndrome in children and is the third most common cause in adults, after focal segmental glomerulosclerosis and membranous glomerulopathy.  A majority of cases are “primary”, require no additional workup, and are due to a circulating permeability factor.   Several possible secondary causes have also been identified.  Although these are rare, these should be considered in...

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...

Here Comes Another Bride

There must be something in the Spring air.  A second bride-to-be on our transcription team was showered with gifts today!  Best wishes to Hillary and her groom!