by Dr. Mahmoud Said

1. Case study
Mahmoud said

2.  A 30 years old lady referred to the renal clinic complaining of
L.L is free except for soft pitting oedema, morning puffiness , and
prot.2+ in urine .
Chest ,heart ,abdomen and LND examination all are free
she is non-diabetic but hypertensive since 5 years
No history of NSAID
 On examination :
B.P:150/100
Pulse:90
L.L edema :2+

3. Which investigations would you ask for
in this case ?????

4.  24hr urinary protein:4g/day
 Urea: 18
 Creat:0.6
 cholest: 350mg/dl
 T.G: 200mg/dl
 S.albumin :2.7
 ESR: 80/120 ????
 ANA ,AntiDsDNA,C3 all are of normal range
 HCVAb , HBSAg negative.
 PT: 100% INR: 1

5. ESR in nephrotic syndrome
The erythrocyte sedimentation rate (ESR) is
elevated (greater than 25 mm/h by the
Westergren method) in almost all patients with
the nephrotic syndrome.(T/F)
This finding alone is not an indication to evaluate a
patient for an underlying systemic disorder (T/F)

6. ESR in nephrotic syndrome
A direct relation between the degree of proteinuria
and the ESR has been noted in patients with
glomerular disease in which the ESR was
approximately 10 times the daily rate of protein
excretion

7. To biopsy or not ?
Biopsy was done

8. Microscopic picture:
with H&E,PAS,trichrome,and congo-red revealed
Widening of the mesangial area-↑glomerular cellularity
+
Thickening of GBM, podocytes were hypertrophied.
Renal tubules-intersetium and the included arterioles were unremarkable

9.  Immunohistochemical for IgG,IgM,IgA
IgG: moderate (++) positive staining in
subendothelial area
IgA: mild (+) positive staining in subendothelial area
IgM:(+/-) positive staining

10.  What is your histopathological diagnosis??
A) membranous GN
B) Mesangioproliferative GN
C) Membranoproliferative Type I
d) Membranoproliferative typeIII
e) non of the above

11. A) Membranous GN
B) Mesangioproliferative GN
C) Membranoproliferative Type I
d) Membranoproliferative typeIII
e) non of the above

12. Well
done

13. Microscopic picture:
with H&E,PAS,trichrome,and congo-red revealed
Widening of the mesangial area-
↑glomerular cellularity
+
Thickening of GBM, podocytes were
hypertrophied.
Renal tubules-intersetium and the included arterioles were
unremarkable

14.  So Why is type I not III?
Immunohistochemical for IgG,IgM,IgA
IgG: moderate (++) positive staining in
subendothelial area
IgA: mild (+) positive staining in subendothelial
area
IgM:(+/-) positive staining

16. EM in Renal Biopsy (KDIGO
guidelines)
sufficient tissue is needed to perform not only an
examination by light microscopy, but also
immunohistochemical staining to detect immune
reactants (including immunoglobulins and
complement components),and
electron microscopy to define precisely the location,
extent and, potentially, the specific characteristics of the
immune deposits. We recognize that electron
microscopy is
not routinely available in many parts of the world, but
the
additional information defined by this technique may
modify
and even change the histologic diagnosis, and may
influence
therapeutic decisions; hence, it is recommended

17.  So you now Know this is a case of
MPGN type I
What will you do ? Based on Evidence !!!!!!

18.  She was put on
ARBs & ACEI
Omega 3 plus ????
Cyclosporin 50 mg 1*2 ???????????
Steroids 40 mg/day with tapering the dose

19. 24 hr.urinary s.creatinine
Date protein g/day s.albumin g/dl mg/dl C0
1.4.2011 4 2.7 0.6
1.6.2011 5 1 88
1.7.2011 6 3 0.7 142
1.8.2011 14 0.6
1.9.2011 3 0.7
1.10.2011 5.2 3.5
1.11.2011 1.3 4 139
1.12.2011 1.2
1.1.2012 0.77
01-02-12 1.5 4.4 1.4 238
1.3.2012 1.8 1.3
28.3.2012 4
1.4.2011 2.6 1.2
25.4.2011 0.8 4.5 1.1
1.6.2011 1.8 1
1.7.2011 1.3
1.8.2011 4 0.9
1.9.2011 1.3
1.10.2011 2.7 3.9

21. During the period of treatment the patient suffered
from severe headache that was not responsive
to any line of treatment she was refereed to a
neurologist . .
A CT scan was done but was unremarkable
MRI was also unremarkable
Bilateral papilledeoma was noted on FUNDUS
examination and was diagnosed as pseudo tumor
cerebri
Acetazolamide and thiazide diuretics was added
along with increasing the dose of the steroids.
She had maevellous response and know she is

22. Prognosis in Idiopathic MPGN
Idiopathic MPGN in adults also carries
an unfavorable prognosis. Five years after biopsy,
50% of patients either die or need renal
replacement therapy (dialysis or transplantation).
This proportion increases to 64% after 10 years.
Risk of progression increases with
elevated creatinine, nephrotic proteinuria, and
severe hypertension or if a biopsy specimen
shows more than 50% crescents or marked
interstitial fibrosis

23. So ,
after this what do you think the prognosis of this
patient is?

24. Do you consider shifting cyclosporin to other
lines of
immunosuppressive drugs is useful or not
?
and Why?

25. Thank you