GN Flashcards
(49 cards)
What are the clinical criteria for nephrotic syndrome?
- > 3 g protein a day
- Hypoalbuminaemia
- Oedema
What are the clinical criteria for nephritic syndrome?
- Proteinuria
- Haematuria
- Hypertesnion
Causes of Nephrotic syndrome?
FSGS
Membranous
Minimal change
Causes of Nephritic syndrome?
IgA nephropathy (focal proliferative)
Membranoproliferative GN
Post strep GN (Diffuse proliferative)
RPGN ( sort of falls within this category)
Causes of RPGN
Anti GBM - (good pastures if lungs involved) Pauci Immune GN - Granulomatosis with polyangitis - Microscopic polyangitis - Churg Straus Immune complex disease - much less common - SLE - IGA - Post strep
Causes of FSGS?
Primary
Secondary
- HIV
- Reflux nephropathy
- Heroin
- Sickle cell
- Alports
- Obesity
Natural History of FSGS?
Progressive CKD is common > 50% progress to ESKD in 10 years
High recurrence in transplants
most common cause of Nephrotic syndrome
Most common GN causing ESKD
What do you see on Biopsy of FSGS?
LM: Scarring/ Scelerosis of some glomeruli (focal) which only affect a portion of the capillary bundle (segmental)
IF: No immune depsoiton
Treatment of FSGS?
ACE/ARB - Pred - 1-2 mg/kg for 3 months then taper if no response - Cyclophosphamide 1-2 mg/kg for 8 weeks If no response - Cyclosporin
Natural history of Membranous GN
Second most common cause of GN More common in people over 50 Untreated - 1/3 recover - 1/3 have variable course - 1/3 have ESKD Most common cause of Renal vein thrombosis in nephrotic syndrome
Pathogenesis of Primary Membranous GN?
Immune complex deposition disease
IgG interacts with PLA2R antigen on the outer aspect of toe glomerular basement membrane
( PLA2R is almost always negative in secondary)
Causes of Secondary Membranous?
Malignancy - solid organ
SLE
HBV - 15-56% of patients !
HCV
Membranous on renal Bx?
LM: Diffuse thickening of the glomerular capillary wall
EM: Electron dense deposits in the subepithelial aspect of the GBM
IF: diffuse granular IgG and C2
Treatment of Membranous?
Given variable course then often can be treated with anti-proteinuria meds and watch
- if nephrotic or proteinuria then treat
no role for steroids alone ( unlike minimal change or FSGS)
1st line
- Cyclophosphamide + cyclical pred
2nd line
- cyclosporin + pred
- ? Rituximab - no RCTS
Anticoagulate in Albumin
What is the pathogenesis of minimal change disease?
Lymphocyte disorder causing t Cells activation and cytokines that alter glomerular capillary perm-selectivity due to loss of electrostatic charge
Natural history of minimal change?
Common in kids
Adults have worse prognosis than kids
Progressive disease is rare
Renal Bx of minimal change disease?
LM: normal
EM: effusion and podocyte effacement
IF: Normal
Treatment of Minimal change?
Steroids - most respond
If no response
- Cyclophosphamide
- Cyclosporin
IgA nephropathy natural history ?
Most common cause of glomerularnephritis
Young age of onset
presents with intermitten haemturia when unwell “pharyngitic”
Frank haematuria = good prognosis
Pathogenesis of IgA?
Abnormal glycosylation of galactose deficient IgA
IgA on renal biopsy ?
LM: Mesangial hypercellularity and matrix expansion
IF: IgA immune deposition
Causes of secondary Membranoprolifertaive GN ?
Secondary Type 1 - 90% - Subendothelial immune deposition - HCV - Cyoglobulinaemia Type 2 - dense deposit disease with reduced serum complement - partial lipodystrophy Type 3 - HBV and HCV
Pathogenesis of primary Membranoproliferative GN?
Due to chronic antigen stimulation causing immune complex formation, also can be due to complement regulation
Natural History of MPGN ?
Poor prognosis
Treat the cause
No benefits of Immunosuppression in adults!
Aspirin + Dipyridamole decrease proteinuria but no effect of GFR
