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Art of Medicine: Minimal Change Disease

Article
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...
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Disease Week: C3 Glomerulonephritis

Article
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...
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Membranous Nephropathy After Transplantation

Article
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...
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Art of Medicine: Lysozyme Nephropathy

Article
The painting above shows acute tubular injury with numerous intracytoplasmic inclusions within proximal tubular epithelium, which can be seen in lysozyme-associated nephropathy.  Lysozyme-induced nephropathy is a rare cause of acute tubular injury seen in patients with hematologic malignancies, most commonly with chronic myelomonocytic leukemia (CMML) and acute monocytic leukemia (Santoriello et al, 2017).   Malignancy associated overproduction of lysozyme can lead to acute kidney injury and proteinuria.  Lysozyme is freely filtered by the glomerulus and reabsorbed by the proximal tubular epithelium.  While this happens at a low level physiologically, over-reabsorption of lysozyme induces toxic acute tubular injury. Lysozyme-induced nephropathy is one...
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Diagnose This (April 22, 2019)

Article
What is this finding and what diagnoses does it imply?       ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​    ...
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Twitter Poll (April 17, 2019)

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ANSWER: D Collapsing glomerulopathy has been associated with certain infectious disease including HIV, Hepatitis C, HTLV-1, parvovirus B19, cytomegalovirus, tuberculosis, Campylobacter enteritis, and Loa loa filariasis. References: Cossey LN, Larsen CP, Liapis H. Collapsing glomerulopathy: a 30-year perspective and single, large center experience. Clin Kidney J 2017; 10(4):443-449 Cohen AH, Nast CC. HIV-associated nephropathy. A unique combined glomerular, tubular, and interstitial lesion. Mod Pathol 1988; 1: 87–97 D’Agati V, Suh JI, Carbone L, et al. Pathology of HIV-associated nephropathy: a detailed morphologic and comparative study. Kidney Int 1989; 35: 1358–1370 Pakasa NM, Nseka NM, Nyimi LM. Secondary collapsing glomerulopathy associated...
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Diagnose This (April 1, 2019)

Article
What is your diagnosis?         ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​...
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Diagnose This (March 25, 2019)

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What is your diagnosis?     ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​ ​  ...
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AL Amyloidosis

Article
A 70 year-old female presents with nephrotic range proteinuria and a creatinine of 1.2. She was in her normal level of health until about 6 months ago when she noticed foamy urine and swelling in her ankles. She has lost 30 lbs unintentionally during the last few months. Her cardiologist said that her proteinuria does not appear to be related to the heart. A kidney biopsy is performed. The glomerulus in figure 1 is distorted and has a “washed-out” or pale appearance on PAS stain. A silver stain shows material which is non-argyrophilic (figure 2). A Congo red stain shows...
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Art of Medicine: Light Chain Proximal Tubulopathy

Article
The painting above shows light chain proximal tubulopathy with crystals, which is one type of a monoclonal gammopathy of renal significance (MGRS).  MGRS, as defined by the International Kidney and Monoclonal Gammopathy Research Group, is a “clonal proliferative disorder that produces a nephrotoxic monoclonal immunoglobulin” (Leung N et al, 2019), and indicates end-organ damage resulting from a monoclonal gammopathy.   The most common MGRS manifestations include AL amyloidosis, monoclonal immunoglobulin deposition disease, light chain cast nephropathy, and light chain proximal tubulopathy.   In light chain proximal tubulopathy, a nephrotoxic monoclonal light chain induces acute tubular injury or proximal tubular dysfunction.  Proximal tubular...
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