MPGN case presentation

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by Dr. Mahmoud Said

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MPGN case presentation

  1. 1. Case study Mahmoud said
  2. 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. 3. Which investigations would you ask for in this case ?????
  4. 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. 5. ESR in nephrotic syndromeThe 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. 6. ESR in nephrotic syndromeA 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. 7. To biopsy or not ?Biopsy was done
  8. 8. Microscopic picture:with H&E,PAS,trichrome,and congo-red revealedWidening of the mesangial area-↑glomerular cellularity +Thickening of GBM, podocytes were hypertrophied.Renal tubules-intersetium and the included arterioles were unremarkable
  9. 9.  Immunohistochemical for IgG,IgM,IgAIgG: moderate (++) positive staining in subendothelial areaIgA: mild (+) positive staining in subendothelial areaIgM:(+/-) positive staining
  10. 10.  What is your histopathological diagnosis??A) membranous GNB) Mesangioproliferative GNC) Membranoproliferative Type Id) Membranoproliferative typeIIIe) non of the above
  11. 11. A) Membranous GNB) Mesangioproliferative GNC) Membranoproliferative Type Id) Membranoproliferative typeIIIe) non of the above
  12. 12. Welldone
  13. 13. Microscopic picture:with H&E,PAS,trichrome,and congo-red revealedWidening of the mesangial area-↑glomerular cellularity +Thickening of GBM, podocytes werehypertrophied.Renal tubules-intersetium and the included arterioles wereunremarkable
  14. 14.  So Why is type I not III? Immunohistochemical for IgG,IgM,IgAIgG: moderate (++) positive staining in subendothelial areaIgA: mild (+) positive staining in subendothelial areaIgM:(+/-) positive staining
  15. 15. EM in Renal Biopsy (KDIGOguidelines) sufficient tissue is needed to perform not only anexamination by light microscopy, but alsoimmunohistochemical staining to detect immunereactants (including immunoglobulins andcomplement components),and electron microscopy to define precisely the location,extent and, potentially, the specific characteristics of theimmune deposits. We recognize that electronmicroscopy isnot routinely available in many parts of the world, buttheadditional information defined by this technique maymodifyand even change the histologic diagnosis, and mayinfluencetherapeutic decisions; hence, it is recommended
  16. 16.  So you now Know this is a case of MPGN type IWhat will you do ? Based on Evidence !!!!!!
  17. 17.  She was put on ARBs & ACEI Omega 3 plus ???? Cyclosporin 50 mg 1*2 ??????????? Steroids 40 mg/day with tapering the dose
  18. 18. 24 hr.urinary s.creatinine Date protein g/day s.albumin g/dl mg/dl C0 1.4.2011 4 2.7 0.61.6.2011 5 1 88 1.7.2011 6 3 0.7 1421.8.2011 14 0.61.9.2011 3 0.71.10.2011 5.2 3.51.11.2011 1.3 4 1391.12.2011 1.21.1.2012 0.7701-02-12 1.5 4.4 1.4 2381.3.2012 1.8 1.328.3.2012 4 1.4.2011 2.6 1.225.4.2011 0.8 4.5 1.11.6.2011 1.8 1 1.7.2011 1.31.8.2011 4 0.91.9.2011 1.31.10.2011 2.7 3.9
  19. 19. 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 unremarkableMRI was also unremarkableBilateral papilledeoma was noted on FUNDUS examination and was diagnosed as pseudo tumor cerebriAcetazolamide and thiazide diuretics was added along with increasing the dose of the steroids.She had maevellous response and know she is
  20. 20. Prognosis in Idiopathic MPGNIdiopathic MPGN in adults also carriesan 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
  21. 21. So ,after this what do you think the prognosis of this patient is?
  22. 22. Do you consider shifting cyclosporin to otherlines of immunosuppressive drugs is useful or not? and Why?
  23. 23. Thank you

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