arrow-right-realarrow-rightarrow-rightback-to-topdoctordownloadfacebookinstagramlogologo-wordmarkcasttwitter
Close Modal

Blog


Currently filtering by tag: Fibrillary glomerulopathy

Twitter Poll (January 9, 2019)

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 (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...

Fibrillary Glomerulopathy

Commonly heard after we give the diagnosis: “I heard about this in Fellowship”… This 62-year-old African-American just completed treatment for hepatitis C with HARVONI® (ledipasvir 90 mg/sofosbuvir 400 mg). He was found to have an elevated creatinine of 1.8 mg/dl (baseline 1.1 mg/dl). Urinalysis showed 3+ protein with no blood and no casts. He also has well-controlled hypertension for more than 10 years. 1. Glomerulus with diffuse mesangial expansion (PAS x400) 2. IgG predominantly mesangial but with extension into loops (200x) 3. Mesangial and loop fibrillary deposits (EM 4,000x) 4. Mesangial and loop fibrillary deposits (EM 12,000x.) Diagnosis: Fibrillary Glomerulopathy