- Randomly arranged fibrils 12-30nm in diameter
- Usually polyclonal IgG
- Presents usually with proteinuria. 50% have nephrotic syndrome at presentation.
- 50% of patients progress to ESRD within a few years
- Treatment often unsuccessful. Steroids, cytoxan, and rituxan have been used with varying success.
- Does not usually recur in transplants
- parallel deposits of straight, hollow-lumen fibrils (microtubules) >30nm in diameter
- usually monoclonal IgG
- usually secondary
- Deposits occur on subepithelial surface of basement membrane, can be indistinguishable from membranous on light microscopy
- Presents with nephrotic syndrome, hypertension, hematuria.
- Recurs in transplants
- randomly arranged curved microtubules 25-35nm in diameter
- 10-15nm fibrils
- Hereditary, autosomal dominant
- Presents with proteinuria, hematuria, hypertension
- Banded collagen fibrils
- Hereditary, autosomal recessive, most cases from Japan
- Presents with edema and nephrotic syndrome
- Progresses to ESRD
For the boards, it may be helpful to remember that the fibril diameter of the more common fibrillary diseases- amyloid, fibrillary GN, and immunotactoid GP are correlated with their position in the alphabet- (A= 8-12nm, F= <30nm, usually 18-20, and I= >30nm, usually 45-55nm).