membranoprolferative gn
DESCRIPTION
MPGN, MCGN presentation, diagnosis and update managementTRANSCRIPT
Membranoprolferative GN
Occur primarily children & young adults.Definition is based on :Mesangial & endothelial cell proliferationExpansion of mesangial matrix Thickened peripheral capillary wallMesangial interposition into the Cap wall
Subendothelial deposits characteristic of membranoproliferative glomerulonephritis Type I
MPGN I
Silver stain
Mesangial proliferation and mesangial interposition beneath the capillary loop endothelial cells with formation of "double contours"
MPGN I
Electron microscopy
The deposits are subendothelial and mesangial. There is duplication of the capillary loop basement membrane between the deposits and interposed mesangium, and the endothelial cells
MPGN I
Direct immunofluorescence
Subendothelial and mesangial deposits of IgG and C3
MPGN II
Electron microscopy
The capillary loops contain interrupted linear electron densities in the subendothelial aspect of the basement membrane.
MPGN II
Direct immunofluorescence
There is bright peripheral loop staining with antibody to C3 as well as mesangial staining. Staining for immunoglobulins is often less intense
MPGN III
Electron microscopy
There are prominent mesangial, subendothelial, and subepithelial electon dense deposits
Mesangial expansion and proliferation
Thickend membrane
Primary or secondary which is more common.
Idiopathic type : I , II , III depending on: IF staining ,ultrastructural appearance & complement profiles.Hypocomplementaemia is characteristic due to decrease synthesis & consumption.
Type I
- Diagnosis by exclusion. - There is discrete deposition in the
mesangium & subendothelial space.
- NS progressive ESRD 50%,90%in10,20ys
- non NS 85% renal survival at 10ys
- 30 – 70 % recurrence in RXT
Type II Dense deposits disease
Deposition along the memb, tubules & boman`s capsule.
- IF +ve for C3 –ve for Ig & complexes- Tram track C3 deposition - High rate of recurrence in RXT 50 – 100 %
Type III
Imcomplex disease.
** C3,C5 & properdin deposition.
** Recurrence is unknown in RXT
Presentation
Asymptomatic proteinuria & haematouria 20 – 30 %NS 40 – 67 %Acute nephritic syndrome 16 – 30 %Gross haematouria 10 – 20 %Azotaemia
Diffuse glomerulonephritis
Inflamatory cells infiltrate in glomeruli & interstitium
Treatment
Reserved for those with:
– proteinuria >3gm/day
- interstitial disease
- impaired renal function
Diet
Normal renal function :normal protein 1 gm/kg/day +urine lossRenal impairment :0.65 – 0.8 gm/day + urine loss Low cholesterol diet
Non specific treatment
BP control Oedema Hyperlipidaemia < 100 mg/dl LDLInfection Thromboembolism
Drug treatment
Prednisolone :2 mg/kg every other day for 1yr then tapered to maintenance of20 mg every other day for 3 – 10 ys120 mg on alternate days for12–16 /52 with follow up .After response taper to 20 – 30 mg alternate days for several ys.
Antiplatelets : Can slow the progression
*-* Aspirin 975mg/day + dipyridamol 225 mg/day OR
*-* Asprin 500 mg/day + dipyridamol 75 mg/day for 3ys.
Cytotoxics : It is not felt that addition of
this group would provide further benefits.