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Disease Week: Membranous Glomerulopathy

Membranous glomerulopathy: Looking forward Many excellent reviews are available that detail the current state of knowledge in membranous glomerulopathy so I will not attempt to round out this #DiseaseWeek by writing another. Instead, I would like to paint, with broad brush strokes, what I believe to be the near future of diagnostics in membranous glomerulopathy and discuss the techniques of discovery being applied today that are likely to similarly impact other types of immune complex-mediated glomerulonephritis. We live in exciting times for discovery in medicine as the tools available today are so powerful that the molecular pathogenic mechanisms of disease...

Art of Medicine: Membranous Glomerulopathy

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...

PLA2R Tutorial

The diagnosis of membranous glomerulopathy has been transformed in the past 10 years with the discovery of PLA2R. PLA2R is the most common target antigen in cases of primary membranous glomerulopathy. Immunohistochemical staining for PLA2R (shown here) can detect the PLA2R type of membranous with high sensitivity and specificity. Recent studies have shown that serum testing for PLA2R antibodies can serve as a useful biomarker for monitoring the clinical activity of this disease. 

PLA2R

Autoantibody formation directed against PLA2R1 is the underlying etiology in most cases of primary membranous glomerulopathy. This new understanding of the pathogenesis of primary membranous has rapidly transformed diagnosis and monitoring of this disease. Proteinuria can take months to resolve after a patient has undergone serologic remission and, as a result, is not an ideal biomarker of disease activity. Testing for levels of serum PLA2R autoantibodies gives a more accurate picture of the patient’s current disease status. Ideally, serum PLA2R testing would be performed immediately after biopsy diagnosis in cases with positive PLA2R staining on biopsy. At this point in...