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Currently filtering by tag: Fibrillary glomerulopathy

Diagnose This (August 3, 2020)

Fibrillary Glomerulopathy, Diagnose This!, Arkana Laboratories, pathology, nephropathology, renal pathology
What is your leading diagnosis when seeing this interesting IgG staining pattern by IF?     ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​...

Disease Week: Fibrillary Glomerulopathy

Fibrillary Glomerulopathy, renal pathology, kidney disease
Monday Did you know Fibrillary Glomerulopathy was described in the 1980s? While case reports existed before, in 1983 a series describing an unnamed renal disease was published. In 1987, the name fibrillary GN was coined in a second small case series. https://www.ncbi.nlm.nih.gov/pubmed/6359891 https://www.ncbi.nlm.nih.gov/pubmed/3106698 Tuesday  Fibrillary GN has an overall poor prognosis. However, more recent studies stratify this more with somewhat consistent percentages of responders, progressive disease, and progression to ESKD. https://www.ncbi.nlm.nih.gov/pubmed/28161700 https://www.ncbi.nlm.nih.gov/pubmed/21441134 Wednesday Rituximab has become a common tx in fibrillary glomerulopathy. Data comes from small, uncontrolled case series but it appears to produce renal response in a subset of...

Twitter Poll (January 9, 2019)

DNAJB9 histo stain, renal pathology, arkana laboratories, pathology
ANSWER: C DNAJB9 immunohistochemical (IHC) stain is a quick and inexpensive tool used to confirm the diagnosis of Fibrillary GN. DNAJB9 IHC has a sensitivity of 98% and >99% specificity.   In 2017, Dr. Nasr et al. discovered a novel proteomic biomarker for Fibrillary GN called DnaJ homolog subfamily B member 9 (DNAJB9), which is a member of the chaperone gene family. DNAJB9 immunohistochemical (IHC) stain is a quick and inexpensive tool used to confirm the diagnosis of Fibrillary GN. DNAJB9 IHC has a sensitivity of 98% and >99% specificity.   Reference: Nasr S, et al. DNAJB9 is a specific...

DNAJB9 helps uncover dual pathology…

This case illustrates the utility of immunohistochemical staining for DNAJB9, a recently described biomarker for fibrillary glomerulopathy (see reference).  The renal biopsy is taken from a 68-year-old man with a history of cirrhosis (cause unknown) who presents with mild microscopic hematuria, non-nephrotic range proteinuria, and chronic renal failure.  Glomeruli show diffuse mild mesangial matrix expansion.  Immunofluorescence shows granular mesangial IgA (2+) and smudgy mesangial and capillary wall IgG (2+) staining.  Electron microscopy (not shown) shows both immune complex-type and fibrillary deposits involving glomerular capillary basement membranes and mesangium.  Immunohistochemical staining for DNAJB9 shows mesangial staining.  Although IgA immunofluorescence staining may...

Crescentic Fibrillary Glomerulopathy

Fibrillary glomerulopathy is a rare disorder of unknown etiology and pathophysiology, which is characterized by deposition of course, randomly-oriented, non-branching fibrils within the mesangium and/or capillary loops. These deposits typically show positive “smudgy” glomerular staining for IgG, C3, kappa and lambda by immunofluorescence. The pattern of glomerular injury by light microscopy is variable; however, mesangial matrix expansion by a PAS-pale, weakly argyrophilic material is almost always present. Cellular crescents (Fig 1 and 2), although uncommon, have been described in up to 17% of cases (see reference).  Given the rarity of this pattern of glomerular injury, its clinical significance is still...

Diagnose This! (June 4, 2018)

The patient is a 67 y/o Caucasian female with slowly progressive kidney disease and 2.3 gm/24 hr proteinuria. Aside from hypertension, she has no significant past medical history. Congo red was negative, however, immunofluorescence showed IgG and lambda staining within glomeruli. What is this immunoperoxidase stain? For bonus points, what was found on electron microscopy?   ​   ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​  ...

Diagnose This (March 12, 2018)

What is your diagnosis?   ​   ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​ ​...

Pushing Glass (August 8, 2017)

A 69-year-old man with past medical history of hypertension presents with nephrotic syndrome and 7 gm/day proteinuria. Serological studies are negative for hepatitis B and C. The images are characteristic findings of which of the following: 1. Amyloidosis 2. Fibrillary Glomerulopathy 3. Light chain deposition disease 4. Diabetic nephropathy 5. Fibronectin glomerulopathy This is a case represents classic findings of fibrillary glomerulopathy. Fibrillary glomerulopathy has been found to be associated with hepatitis C infection, malignancy, dysproteinemia and autoimmune disease (see References below). The deposits in fibrillary glomerulopathy are weakly PAS-positive, silver negative and Congo red negative. By electron microscopy, there...