FIBRILLARY GN & IMMUNOTACTOID GN Flashcards
What is the size of fibrils in Fibrillary GN?
16-24 nm (mean: 20nm)
What is the size of fibrils in Immunotactoid GN?
• 30-50 nm
• hollow microtubules
What is a a specific immunohistochemical marker found in the distribution of the fibrillary glomerular deposits?
DNAJB9
What is a rare form of fibrillary renal disease in which the glomeruli are infiltrated by massive deposits of fibronectin?
FIBRONECTIN GLOMERULOPATHY
What type of GN occurs mostly in adults, in both sexes & in all age groups?
FIBRILLARY GN
What are the 3 disorders associated with Fibrillary GN?
- MALIGNANCY
- MONOCLONAL GAMMOPATHY
- AUTOIMMUNE DISORDERS
Patients with this type of fibrillary GN tend to be older, may have a less rapidly progressive course
IMMUNOTACTOID GN
What disorder is associated with Immunotactoid GN?
LYMPHOPROLIFERATIVE DISORDERS
What infection is associated with Fibrillary & Immunotactoid GN?
HCV
What are the pathognomonic EM findings in Amyloid, Fibrillary & Immunotactoid GN?
AMYLOID: 8-12 nm
FIBRILLARY: 16-24 nm, non-branching
IMMUNOTACTOID: 20-30 nm, hollow
What type of GN where fibrils are arranged randomly in the mesangial matrix and GBMs?
FIBRILLARY GN
What type of GN where the microtubules are often arranged in parallel stacks in the mesangium, subendothelial, and/or subepithelial regions?
IMMUNOTACTOID GN
What is the IF staining in Fibrillary GN?
• POLYCLONAL
• always for IgG (IgG1 and IgG4)
• also C3, and both κ and λ chains,
What is the 2 most common IgG subclass in Fibrillary GN?
• IgG1
• IgG4
What is the IF staining in immunotactoid?
• MONOCLONAL
• IgG
What are the 2 most common IgG subclass associated with Immunotactoid GN?
• IgG1
• IgG2
In both Fibrillary and Immunotactoid GN, deposits are limited to where?
GLOMERULUS
What are the 2 treatments used for fibrillary GN with crescentic pattern on LM?
- CORTICOSTEROIDS
- CYCLOPHOSPHAMIDE
What is the treatment used for fibrillary GN with membranous pattern on LM?
CYCLOSPORINE
What is the treatment used for fibrillary GN with MPGN pattern on LM?
RITUXIMAB
What is the type of Fibrillary Renal Disease which is a familial disease with autosomal dominant inheritance that presents with proteinuria and hematuria usually in adolescents and eventually progresses to nephrotic syndrome and slowly deteriorating renal function?
Fibronectin glomerulopathy
Fibronectin glomerulopathy is caused by an inherited mutation in the gene encoding?
FIBRONECTIN-1
