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.