Case Presentation Turki Al-Hussain, MD

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1 Case Presentation Turki Al-Hussain, MD Director, Renal Pathology Chapter Saudi Society of Nephrology & Transplantation Consultant Nephropathologist & Urological Pathologist Department of Pathology & Laboratory Medicine King Faisal Specialist Hospital & Research Centre 1

2 Clinical History An 8 year-old boy presented with vomiting and dark urine H/O sore throat H/O deftness in two of his cousins Family history of renal disease Physical examination: Temperature: 36.6 C, respiratory rate: 22/MIN, blood pressure: 106/70 mmhg and heart rate: 128/MIN Urine dipstick: RBC 3+, protein 2+ 2

3 Laboratory Data Urinalysis: 3+ blood, 3+ protein, 5-10 WBC/HPF, numerous RBC with RBC cast. Urine culture: no growth. ANA, canca, panca: Normal Throat culture: Negative Serum creatinine: 701 μmol/l (baseline 39) HIGH Complements: C3: 0.11g/l (n ) LOW C4: 0.26 g/l (n ) 3

4 Rapidly Progressive Glomerulonephritis 4

5 Renal Biopsy was Performed 5

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18 Acute Diffuse Proliferative Glomerulonephritis with 50% Cellular Crescents 18

19 Differential Diagnosis Acute postinfectious glomerulonephritis Membranoproliferative glomerulonephritis type 1 Dense deposit disease (C3 glomerulopathy) Lupus nephritis IgA nephropathy 19

20 MPGN type 1 IgG C3 20

21 Dense deposit disease C3 21

22 Lupus nephritis Ig & C 22

23 IgA nephropathy IgA 23

24 Immunofluorescene IgG IgA IgM C1q Fibrinogen Kappa light chain Lambda light chain ALL NEGATIVE 24

25 IF: C3 25

26 IF: C3 26

27 ??? C3 Glomerulopathy 27

28 EM 28

29 EM 29

30 EM 30

31 EM 31

32 EM 32

33 Additional Laboratory Data: HIGH Anti-streptolysin O (ASO) titer: 454 IU (normal < 200 IU) 33

34 Final Diagnosis Acute Postinfectious Glomerulonephritis with 50% Cellular Crescents 34

35 Should IgG always present in acute postinfectious glomerulonephritis? No Some cases may have only C3 35

36 Follow up Peritoneal Dialysis IV Augmentin Pulse methylprednisolone for five days and then was switched to oral prednisolone 45 mg O.D, then tapered down. On discharge serum creatinine became 34 μmol/l C3 became normal after 1.5 month: 1.35 g/l(n ) 36

37 C3 Glomerulopathy A disease process due to abnormal control of complement activation, deposition, or degradation and characterized by predominant glomerular C3 fragment deposition with electron-dense deposits on EM. Based on EM appearances, it is divided into: 1- Dense Deposit Disease (DDD) 2- C3 Glomerulonephritis (C3GN) 37

38 The evolving classification of MPGN D'Agati VD et al. C3 glomerulopathy: what's in a name? Kidney Int ;82:

39 Why C3 Glomerulopathy was introduced? 1. The glomerular pathology associated with isolated C3 accumulation is far more heterogeneous than previously appreciated. 2. Advances in our understanding of complement-mediated kidney injury have made it possible to identify the cause of renal disease through specific complement investigations. 3. The existence of a licensed complement inhibitor (eculizumab) together with the many complement inhibitors in clinical development, so patients most likely will benefit from an anti-complement therapeutic approach. 39

40 C3 Glomerulopathy Glomerulonephritis with dominant C3 should be used as a morphological term for those cases with dominant staining for C3c. Dominant is defined as C3c intensity 2 orders of magnitude more than any other immune reactant on a scale of 0 to 3 (including 0, trace, 1+, 2+, 3+). 40

41 Pickering MC et al: C3 glomerulopathy: consensus report. Kidney Int. 2013; 84:

42 Gale DP, Pickering MC. Regulating complement in the kidney: insights from CFHR5 nephropathy. Dis Model Mech. 2011; 4:

43 Light Microscopic Features Mesangial proliferative Membranoproliferative Endocapillary proliferative Crescents may also be present In rare cases, glomeruli may be normal by LM 43

44 44

45 Postinfectious GN The differentiation of true PIGN from C3 glomerulonephritis often cannot be made on the basis of morphology and clinical and laboratory data available at the time of biopsy. According to the recommendations, a PIGN patient s biopsy may be read as glomerulonephritis with dominant C3 (infection-associated). However, this does not mean that the patient has C3 glomerulopathy. 45

46 Postinfectious GN In these cases, refining the differential diagnosis will require following the patient clinically and serologically over several months to determine the course of urinary abnormalities and serum C3 levels. If these parameters do not follow a typical course of PIGN (i.e., normalization of the decreased peripheral C3 level in 8 12 weeks), a diagnosis of C3 glomerulopathy should be reconsidered and additional investigations performed. 46

47 Pickering MC et al: C3 glomerulopathy: consensus report. Kidney Int. 2013; 84:

48 Patients with DDD were younger, more likely to have low serum C3 levels, and more likely to have crescentic GN than patients with C3GN. Patients with C3GN were older and had more severe arteriolar sclerosis, glomerular sclerosis, and interstitial scarring on renal biopsy than patients with DDD. Older age, crescentic GN, and DDD subtype were independent predictors of ESRD. Renal impairment at the time of renal biopsy predicted a poor prognosis only in patients with DDD. 48

49 Acknowledgment Dr. Fayzeh Mansour ( Pediatric Nephrologist at Security Forces Hospital) 49

50 References 1. Pickering MC et al: C3 glomerulopathy: consensus report. Kidney Int. 2013; 84: Medjeral-Thomas NR et al: C3 glomerulopathy: clinicopathologic features and predictors of outcome. Clin J Am Soc Nephrol. 2014;9: Hou J et al: Toward a working definition of C3 glomerulopathy by immunofluorescence. Kidney Int Sep 25. [Epub ahead of print] 4. Barbour TD, Pickering MC, Cook HT. Recent insights into C3 glomerulopathy. Nephrol Dial Transplant. 2013;28: D'Agati VD, Bomback AS. C3 glomerulopathy: what's in a name? Kidney Int. 2012; 82: Gale DP, Pickering MC. Regulating complement in the kidney: insights from CFHR5 nephropathy. Dis Model Mech. 2011; 4:

51 THANK YOU 51

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