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Twitter Poll (October 14, 2020)

Exostosin, arkana laboratories, autoimmune diseases, renal pathology
Answer: D Exostosin is an antigen that was recently identified out of a cohort of Phospholipase A2 Receptor (PLA2R) and Thrombospondin Type-1 Domain-Containing 7A (THSD7A) negative cases of Membranous GN. Results showed that EXT1/2 positive cases were associated with autoimmune disease, including systemic lupus erythematosus (see reference).   Reference: Sethi S, Madden BJ, Debiec H, et al. Exostosin 1/Exostosin 2-Associated Membranous Nephropathy. J Am Soc Nephrol 2019; 30(6): 1123-1136.  

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.  

Disease Week: Membranous Glomerulopathy

Disease Week, Membranous glomerulopathy, disease week, arkana laboratories
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

KDIGO, Membranous Glomerulopathy, KDIGO Connections, renal pathology
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!  

THSD7A

Granular capillary loop staining for thrombospondin type-1 domain-containing 7A (THSD7A). Our understanding of the pathogenesis of membranous glomerulopathy was transformed with the discovery that most cases of ‘idiopathic’ MN are due to autoantibodies directed against the phospholipase A2 receptor (PLA2R) protein.(1) More recently, thrombospondin type-1 domain-containing 7A (THSD7A) protein was described as a second antigenic target of autoantibodies in this disease.(2) Recent reports have implicated malignancy as the underlying etiology in some cases of THSD7A-associated MN.(3-5) 1. Beck LH, Bonegio RG, Lambeau G, et al. M-type phospholipase A2 receptor as target antigen in idiopathic membranous nephropathy. N Engl J Med...