1. 사구체신염 진단 Tips 신장내과 임천규

2. Chang JH et al, Nephrol Dial Transplant 2009 Changing prevalence of glomerular diseases in Korean adults IgAN MN MCD FSGS MPGN

3. Glomerular diseases Tubulointerstitial diseases Vascular diseases DDx

4. Glomerular Disease Primary Secondary Associated with systemic disease

5. Acute GN, acute nephritic syndrome Nephrotic syndrome Rapidly progressive GN (crescentic GN) Asymptomatic urinary abnormalities Chronic GN Glomerular Disease (5 major syndrome)

6. Glomerular syndrome (Harrison) Acute Nephritic Syndrome Pulmonary-Renal Syndrome Nephrotic syndrome Basement Membrane Syndrome Glomerular Vascular Syndrome Infectious Disease–Associated Syndrome

7. 신염 Nephritic pattern 신증후군 Nephrotic pattern Mixed syndrome Isolated proteinuria or hematuria Glomerular Disease

8. Locus of immune deposition Linear deposition: Anti-GBM disease Granular deposition: Subepithelial: PSGN, MGN Subendothelial: MPGN, Lupus N (IV) Mesangial: IgAN, HSP, Lupus N(II)

9. Nephritic Syndrome  Hematuria (RBC cast)  Proteinuria, non-nephrotic  Oliguria  Renal insufficiency  Edema & hypertension

10. Postinfectious GN Lupus nephritis IgA nephropathy & Henoch-Schönlein purpura Anti-GBM disease ANCA small-vessel vasculitis Cryoglobulinemia MPGN Granulomatosis with polyangiitis (Wegener's) Microscopic polyangiitis Churg-Strauss syndrome PSGN Subacute bacterial endocarditis Shunt Visceral abscess Nephritic Syndrome

11. Nephritic syndrome and age <15 years – PIGN, MPGN 15 to 40 years – PIGN, lupus N, crescentic GN, IgAN, MPGN >40 years – crescentic GN, vasculitis, IgAN, PIGN

12. Serologic testing in Nephritic syndrome Antineutrophil cytoplasmic antibody (ANCA) Anti-streptococcal antibodies Anti-GBM Cryoglobulin HBV HCV

13. Hypocomplementemia C3↓ PSGN Type I, II MPGN (C3 GN) Lupus nephritis Cryoglobulinemia

14. Gross hematuria IgA nephropathy PSGN (^adult; staphylococcal infection) Alport’s syndrome Thin basement membrane disease <10% Type 2 MPGN or C3 GN Anti-GBM disease Lupus nephritis

15. Hematuria after URI IgA nephropathy PSGN Alport’s syndrome Thin basement membrane disease MPGN or C3 GN

16. 남자 17 세, 혈뇨, proteinuria, SCr 2.0 mg/dL C3

17. 여자 23 세 SCr 1.0 mg/dL, U/A: RBC 10-20/hpf, proteinuria 1.0 g/d IgA

18. IgA nephropathy vs PSGN Less than five days vs 1-3 weeks after URI Throat culture and ASO test Serum C3 Recurrent episodes of gross hematuria Chronic disease and persistent microscopic hematuria

19. IgAN: Clinical presentation Recurrent macroscopic hematuria Slowly progressive IgAN Microscopic hematuria & little proteinuria Crescentic IgAN IgA nephrosis

20. Acute GN and pulmonary hemorrhage ANCA-positive vasculitis: Granulomatosis with polyangiitis Microscopic polyangiitis Anti-GBM antibody disease (G-P syndrome) Rare in lupus, HS Purpura, --

21. 여자 66 세 SCr 2.5 mg/dL, U/A: RBC 10-20/hpf, proteinuria 1.7 g/d

22. Anti-GBM disease Immune complex GN Pauci-immune GN Linear deposit Granular No deposit Anti-GBM Ab Serum C3 ANCA G-P syndrome Anti-GBM GN Granulomatosis with polyangiitis Microscopic polyangiitis Renal-limited crescentic GN Crescentic GN

25. 남자 18 세 단백뇨 1 일 14g, 혈청 알부민 2.0g/dL Sudden onset of nephrotic syndrome (1-2wk)

26. Diffuse effacement of the podocyte foot processes on electron microscopy

27. ■ Heavy proteinuria > 3.5 g/day  hypoalbuminemia  edema  hypercholesterolemia  lipiduria Nephrotic Syndrome

28. Minimal change disease Focal segmental glomerulosclerosis Membranous nephropathy Diabetic nephropathy Amyloidosis Pure Nephrotic Syndrome Abrupt onset of NS 혈뇨 ?

29. Nephrotic syndrome and Age < 15 years – MCNS, FSGS, MsPGN 15 to 40 years – MCNS, FSGS, MN, DN > 40 years – FSGS, MN, DN, MCNS, primary amyloidosis (> 60 yrs), benign nephrosclerosis

30. Serologic testing in Nephrotic syndrome Antinuclear antibodies (ANA), anti-double-stranded DNA antibodies hypocomplementemia  LN Monoclonal gammopathy  Primary amyloidosis and light chain deposition disease HBV  MN, MPGN HCV infection  MN, MPGN Syphilis  MN HIV  Collapsing FSGS

31. 남자 32 세 단백뇨 1 일 2.0g U/A RBC 0-2/hpf Minimal change disease?

32. 여자 47 세 단백뇨 1 일 3.5g, 혈청 알부민 3.0g/dL U/A RBC 5-9/hpf

33. FSGS vs MCD Clinical features that are more common in FSGS are hematuria, hypertension, and decreased renal function.

34. 여자 17 세 단백뇨 1 일 5.0g Pathology: diffuse foot process effacement  4mo steroid Tx 에도 무반응  Rebiopsy 상 FSGS

35. MCD vs FSGS Primary FSGS: segmental glomerulosclerosis in at least one glomerulus in addition to diffuse foot process effacement Incorrect diagnosis of MCD: sclerotic changes occur first in the juxtamedullary glomeruli

36. Diagnostic concerns in FSGS Sampling error Distinguishing primary and secondary FSGS

37. FSGS Primary FSGS 2ndary FSGS  An adaptive response to glomerular hypertrophy and hyperfiltration,  Scarring due to previous injury  Direct toxic injury to podocytes

38. Primary vs Secondary FSGS Primary FSGS – acute onset of the nephrotic syndrome Secondary FSGS –slowly increasing proteinuria and renal insufficiency –often non-nephrotic –nephrotic range proteinuria & hypoalbuminemia (-) Edema (-) –Focal foot process effacement

39. Serum creatinine ↑ in Nephrotic syndrome Minimal change nephrotic syndrome –Prerenal –Interstial edema –Ischemic ATN –MCD with acute interstitial nephritis by NSAID Collapsing form of FSGS Membranous nephropathy –superimposed crescentic GN –Renal vein thrombosis

40. 여자 59 세, Proteinuria 4.8g/day

41. Membranous nephropathy Beck LH, Kidney Int 2010

42. 57 세 여자, Urinalysis albumin (+/-) 24hr urine protein 7.0g

45. Nephrotic and nephritic Postinfectious glomerulonephritis MPGN IgA nephropathy Lupus N

46. C3 16 mg/dL, C4 20 mg/dL 남자 16 세 SCr 1.0 mg/dL, U/A: RBC 10-20/hpf, proteinuria 3.7 g/d

47. 여자 17 세 U/A: RBC 10-20/hpf, urine protein 4.8 g/d serum Cr 1.8 mg/dL

48. Lupus Nephritis (ISN/RPS 2003)

50. Diffuse proliferative GN

51. Glomerular hematuria Significant proteinuria Dysmorphic RBC & acanthocytes RBC cast

52. Isolated glomerular hematuria IgA nephropathy, Alport’s syndrome, Thin BM disease Non-nephrotic proteinuria Benign, functional, transient, orthostatic proteinuria Sustained proteinuria >1–2 g/day: Glomerular disease Diabetic nephropathy, MsPGN Isolated proteinuria or hematuria

53. Adults with microscopic hematuria * Hematuria & proteinuria  increased risk of renal failure * Hematuria & proteinuria  High prevalence of IgAN

56. GN 공통 예후인자들 ? Proteinuria Decreased renal function Hypertension Renal biopsy: glomerulosclerosis, interstitial fibrosis Other: male, old age

58. Stars fade out as the Sun rises.