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Currently filtering by tag: Fibrils

Twitter Poll (September 16, 2020)

Fibrillary glomerulonephritis, arkana laboratories, twitter poll
Answer: C Fibrillary glomerulonephritis is a rare disease affecting less than 1% of all native kidney biopsies and is due to fibrillary deposits of immunoglobulin that by electron microscopy are randomly arranged fibrils of approximately 18-20 nm in diameter. Clinically patients will present with proteinuria and hematuria. Histologically it can show several patterns within glomeruli including mesangial proliferative, membranoproliferative, or even a crescentic pattern of glomerular injury. Positive staining with the DNAJB9 stain is extremely useful in identifying this disease as it is around 98% sensitive and 99% specific for Fibrillary GN. Associations with Fibrillary GN include autoimmune disease, infection...

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

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 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 patients....

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...

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