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Diagnose This (August 17 ,2020)

Membranous Glomerulopathy, PLA2R, Diagnose This, renal pathology, Arkana Laboratories
Q: What is your diagnosis for this patient with nephrotic syndrome?   The electron photomicrograph demonstrates numerous subepithelial deposits along the glomerular basement membrane characteristic of a Membranous Glomerulopathy. Subsequent staining with an immunoperoxidase stain for Phospholipase A2 receptor (PLA2R) was strongly and diffusely positive in this case consistent with a primary Membranous GN. By light microscopy, the classic findings include thickened glomerular basement membranes and pinholes (or bubbles) and spikes that can be seen along the capillary loops with the Jones silver stain. However, it should be noted that the degree, or severity, of these findings can be extremely...

Membranous Glomerulopathy Subtyping

Membranous Glomerulopathy Subtyping, Arkana Laboratories, Renal Pathology Teaching Points
The diagnosis of membranous glomerulopathy has evolved rapidly in the past 10 years since the identification of PLA2R as the major target antigen in primary membranous glomerulopathy. Today, rather than subtyping into vague categories such as 'primary' and 'secondary', we have tools available to name the specific type of disease present in most cases. Arkana Laboratories offers diagnostic testing for membranous glomerulopathy including immunohistochemical stains for tissue typing of PLA2R, THSD7A, EXT1/2, and NELL1 membranous and serum testing to monitor disease in patients with PLA2R and THSD7A-associated membranous glomerulopathy.  

Resolving Membranous Nephropathy

Arkana Laboratories, renal pathology, Membranous Nephropathy
A 40-year-old white male presents with 3 grams/24 hr of proteinuria and swelling around his ankles. He reports that he has had foamy urine over the last 4 months, but was unable to go to a doctor till now because of lack of insurance. Image 1 and image 2 shows irregularly thickened glomerular basement membranes with lamellation. Image 3 shows numerous "holes" on silver stain. Images 4, 5, and 6 show weak staining for IgG, kappa, and lambda, respectively. Membranous nephropathy in a resolving phase can be a tricky diagnosis since the immunofluorescence staining may be extremely weak or negative....

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...

Disease Week: Membranous Glomerulopathy

Monday History of MG Timeline of major discoveries in membranous glomerulopathy.   Heymann nephritis was unique as a model of membranous glomerulopathy when it was reported in 1959 because it was the first to produce renal disease through autosensitization. http://pediatrics.aappublications.org/content/7/5/691 The antigenic target of Heymann nephritis (megalin) is largely absent from human podocytes but does have a role in human autoimmune disease as the antigenic target of anti-brush border antibody disease (LRP2-assoc nephropathy). https://jasn.asnjournals.org/content/29/2/644.long PLA2R was described as the major autoantigen of primary MG in 2009, approximately 50 years after the initial description of this form of glomerulonephritis. https://www.nejm.org/doi/full/10.1056/NEJMoa0810457 Tuesday...

Art of Medicine: Membranous Glomerulopathy

Membranous Glomerulopathy, Art of Medicine
The painting above depicts membranous glomerulopathy.   A single glomerular capillary loop with confluent subepithelial and intramembranous electron dense deposits along the glomerular basement membrane is shown.  Podocytes are showing foot process effacement and microvillous transformation, which results in loss of the filtration barrier leading to nephrotic syndrome.  An electron micrograph from a patient with membranous glomerulopathy is shown below. Membranous glomerulopathy is the second most common cause of nephrotic syndrome in adults.  A majority of cases are considered primary with autoantibodies directed against the podocyte antigens phospholipase A2 receptor (PLA2R, ~70% of cases) or thrombospondin type 1 domain containing 7A...

KDIGO Connections: Membranous Glomerulopathy

Welcome to the first post in our new series KDIGO Connections, a series in which we are asking our nephrologist colleagues to educate us in real-world experiences treating kidney disease. In this series, Dr. David Bourne will be kicking it off with KDIGO guidelines for a kidney disease in visual abstract form. Our hope is that these posts will foster discussion regarding these diseases and how different providers approach them. Please share any points, pearls, questions, caveats, thoughts, or experience you have on the presented disease letting us all learn from your experience!  

Interstitial Foam Cells

This 52-year-old female underwent a renal biopsy as part of the workup for nephrotic syndrome. The biopsy is diagnostic for PLA2R-positive membranous glomerulopathy with mild glomerular and tubulointerstitial chronic injury (not shown). Interestingly, the interstitium shows frequent clusters of interstitial foam cells (see image). While interstitial foam cells are commonly associated with Alport syndrome, they may actually be seen in a wide range of renal diseases resulting in heavy proteinuria, such as focal segmental glomerulosclerosis (FSGS), membranous glomerulopathy, IgA nephropathy and in diseases with a membranoproliferative pattern of glomerular injury. While it has been shown that interstitial foam cells contain...