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Twitter Poll (September 16, 2020)

Fibrillary glomerulonephritis, arkana laboratories, twitter poll
Answer: C Fibrillary glomerulonephritis is a rare disease affecting less than 1% of all native kidney biopsies and is due to fibrillary deposits of immunoglobulin that by electron microscopy are randomly arranged fibrils of approximately 18-20 nm in diameter. Clinically patients will present with proteinuria and hematuria. Histologically it can show several patterns within glomeruli including mesangial proliferative, membranoproliferative, or even a crescentic pattern of glomerular injury. Positive staining with the DNAJB9 stain is extremely useful in identifying this disease as it is around 98% sensitive and 99% specific for Fibrillary GN. Associations with Fibrillary GN include autoimmune disease, infection...

Membranous Lupus Nephritis

Membranous Lupus Nephritis, arkana laboratories, renal pathology, kidney biopsy
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...

Art of Medicine: Collapsing Glomerulopathy

Collapsing Glomerulopathy
The above painting shows a glomerulus with capillary tuft collapse, visceral epithelial cell hyperplasia, and numerous protein resorption droplets within Bowman’s space; findings that can be seen in collapsing glomerulopathy.  A PAS stain from a case of collapsing glomerulopathy is also shown in the photomicrograph below.  Initially, collapsing glomerulopathy was considered a severe form of focal segmental glomerulosclerosis and placed into the Columbia classification. According to the Columbia classification, collapse of at least one capillary loop with obliteration of the lumen and proliferation and hypertrophy of overlying podocytes is sufficient for a diagnosis of the collapsing glomerulopathy.  Tubulointerstitial damage is...

Art of Medicine: Diabetic Nephropathy

Diabetic Nephropathy, Art of Medicine
The above painting shows glomeruli with nodular mesangial expansion and arterial hyalinosis, changes frequently seen in diabetic nephropathy. Diabetic nephropathy is graded by the classification system established by the Renal Pathology Society to separate lesions into varying degrees of severity. In class I diabetic glomerulopathy, there are no changes identified by light microscopy, but thickening of the glomerular basement membranes are seen on electron microscopy (see photomicrograph below). Thickened glomerular basement membranes are greater than 471 nm in women or 520 nm in men (in our lab), which represents greater than 2 standard deviations above the normal population. Glomerular basement...

Twitter Poll (May 22, 2019)

IgA nephropathy, Twitter Poll, Arkana Laboratories, kidney pathology, renal disease
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.    

Art of Medicine: Minimal Change Disease

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, Arkana Laboratories, Disease Week, renal disease, kidney pathology
Monday Rediscovery of Complement Mesangial C3 deposition only within the glomerulus has been reported since 1980. Only recognized recently as a distinct entity when CFHR5 nephropathy was reported. CFHR5 mutation is speculated to represent a founder mutation which can be traced back to the Troodos mountains of Cyprus.   1 in 6500 people in Cyrus carry a mutation in CFHR5 (duplication of exons 2 and 3) and inheritance is autosomal dominant. C3 and C4 complement levels are normal. 80% of males with CFHR5 nephropathy progress to ESRD while only 20% of females progress to ESRD. CFHR5 nephropathy is...

Membranous Nephropathy After Transplantation

Membranous Nephropathy, renal diseases, kidney biopsy, arkana laboratories
The biopsy shows a recurrent membranous nephropathy within a transplant. Figure 1 shows "spikes" and "holes." Figure 2 shows mild interstitial fibrosis. Figures 3, 4, 5, and 6 shows IgG, kappa, lambda, and PLA2r, respectively. Figure 7 shows subepithelial and intramembranous deposits. Membranous nephropathy can arise in the graft from recurrence of the original disease, a de novo disease, and rarely as a donor-derived disease. Recurrence of the original disease can be seen as early as one week post transplant. Most cases of recurrent membranous nephropathy occur within the first few months following transplantation, whereas de novo membranous nephropathy usually...