Glomerular disease & The major glomerulopathy

1. crescentic GN Ұ Ÿ ? parietal epithelial cell

2. ܺ׿ ħϴ ڰü Ű

1) molecular mimickry : ܺ׿ glomerular Ag Ͽ ܺ׿

ü glomerular Ag autoAb ۿ.

2) MHC class II expression : ܺ׿ glomerular cellǥ鿡 MHC class II

expression Ͽ ִ ڰ׿ T cell Ű Ͽ ڰ鿪

۵.

3) polyclonal activation of B lymphocyte

: ܺ׿ B lymphocyte polyclonal activation Ͽ Ϻΰ

nepritogenic Ab .

3. Heymann nephritis: immune glomerular injury ϱ 㿡

IF & EM ΰ MGN ϴ.

4. subendothelial deposit: nephritic type

subepithelial deposit: nephrotic type

=> subepithelial space Abħ proteinuria ϴ.

anionic Ab: GBM negative charge Ƿ subendothelial cell space & mesangium

trap

cationic Ab: GBM Ͽ subepithelial space ħȴ.

5. antiendothelial cell antibody: endothelial antigen circulating antibody

several inflammatory vasculitis & GN Ǿ.

antibody titer disease activity̿ ϴ ִ.

* pathogenic role: endothelial cell activation leukocyte adhesiveness Ŵ

6. glomerular disease ûϴ UAҰ

RBC cast, dysmorphic RBC, large amount of proteinuria

7. RPGN ܿ ʿ serologic markers

ANCA, complement, anti-GBM Ab

8. acute nephritic syndrome

: ARF & oliguria, hematuria, edema & hypertension,

GFR, salt & water tubular reabsorption

RBC cast, dysmorphic RBC

9. complement : ComPAC SLIM(Shunt nephritis, Lupus, Idiopathi MPGN)

PSGN, lupus nephritis, SBE(shunt nephritis), cryoglobulinemia, idopathic MPGN

10. PSGN

Group A -hemolytic streptococcus (throat or skin)

streptococcal exoenzyme Ab: ASO, anti-DNase B, ASKase, anti-NADase,

AHase

C3

cf. Ab 7ĺ Ͽ 1 peak ϸ 3-4 ȭȴ.

11. PSGN poor Px

adult, sporadic, persistent proteinuria, hypertension, severe underlying disease(severe

cresecent), slowly progressive glomerulosclerosis, renal failure

12. primary RPGN з

type I; anti-GBM disease = Goodpasture's disease

type II: immune complex-mediated disease = IgA, MPGN, PSGN, SLE..

type III: Pauci-immune disease = Wegener's, Churg-Strauss, Microscopic polyangitis

13. RPGN ߾(drug hypersensitivity)

R - Rifampin P - penicillamine, phenylbutazone

G - (hydralazine) N- enalapril

14. c-ANCA(+) : Wegener's granulomatosis(=Գʽ ׷θý)

p-ANAC(+) : microscopic PAN, Churg-strauss syndrome

atypical p-ANCA: UC, Crohn's ds, primary sclerosing cholangitis

15. RPGN poor prognostic factor

crescent >70%, oliguria, s-Cr >6 mg/dL,

anti-GBM Ab mediated, severe tubulointerstitial disease

16. plasmapheresis Ǵ ȯ

HUS, RPGN, Goodpasture's syndrome, lupus nephritis

17. Nephrotic syndrome պ

protein malnutrition: vit D deficiency -> hypocalcemia, osteomalacia

hyperlipidemia & accelerated cardiovascular disease: LDL, HDL, T-chol,

lipoprotein

infection risk

thromboembolic tendency

microcytic hypochromic anemia: ö ҽǶ

18. RVT in nephrotic syndrome

ϸ hemorrhagic infarct, renal rupture, hypovolemic shock ų ִ.

MGN, MPGN պ 찡 ϴ.

* hypercoagulable state

i) prothrombin III deficiency

ii) protein C, S, activity

iii) liver fibrinogen synthesis

iv) impaired fibrinolysis

v) platelet aggregation

* RVT ϴ ȯ

MGN, MPGN, amyloidosis

19. NS anticoagulation Ix

DVT(+), arterial thrombosis(+), pulmonary embolism(+)

20. RVT

i) nephrotic syndrome: MGN, MPGN, amyloidosis

ii) RCC invasion

iii) pregnancy or estrogen therapy

iv) volume depletion(infants)

v) extrinsic compression

21. secondary nephrotic syndrome

infection: PSGN, hepatitis B

drug : NSAID

neoplasia, multisystem, DM, chronic allograft rejection

22. ο nephrotic syndrome ? malignancy, amyloidosis

23. NS proteinuria ҽŰ

protein restriction, ACE inhibitor, NSAIDs, low dose aspirin, dipyridamole

24. secondary MCNS

1) systemic ds: Hodgkins' ds & other lymphoproliferative malignancy

DM, Fabry's disease, IgA nephropthy, HIV infection

2) drug: NSAIDs, rifampin, interferon-, heroin, iron dextran

"ٱ(RIND-) heroinϴٰ MCNS ɷȴ"

cf. DM, Fabry's disease, HIV: MCD, FSGS

25. MCD steroid

1) steroid-responsive: ġ 8ֳ proteinuria complete remission

2) steroid-resistant: ġ 8ֳ remission

3) complete remission: urinary protein < 4mg/hr/m2 or 3 dipstick 0-trace

4) steroid-dependent: ó ϳ steroid ̰ų ߴ 2ֳ

5) frequently relapse: ó ϳ 6 2 Ȥ 18 6

26. MCD steroid 8 ġ 1 4ȸ Ͽٸ ġ Ǵ?

frequently relapsing MCNS

-> ġ: cyclophosphamide, chlorambucil

S/E: cystitis, infertility, alopecia, secondary malignancy

27. FSGS

nonselective proteinuria(2/3), hypertension(30-50%), microscopic hematuria(1/2)

GFR(20-30%)

8 ġ remission rate: 20-40%

28. FSGS poor prognostic factor

hypertension, abnormal renal function, black race, persistent heavy proteinuria

29. FSGS ݵǴ ȯ

nephron loss FSGS ִ.

i) reflux nephropathy

ii) GN

iii) interstitial nephritis

iv) sickle cell disease

v) ischemia & cyclosporin nephropathy

vi) renal allograft rejection

vii) heroin abuse, HIV, DM, Fabry's disease, obesity with sleep apnea syndrome

* heroin, HIV, DM, Fabry's disease MCD

* HIV infection ݵǴ GN? FSGS

30. Fibrillary-immunotactoid glomerulopathy

LM: mesangial expansion & BM thickening with PAS(+) material

EM: PAS(+) material ϰ 迭(fibrillary glomerulopathy:18-22 nm)

or organized bundles(immunotactoid glomerulopathy: 30 nm, plasma cell dyscrasia

)

lymphoproliferative malignancy , ġ ȯڰ 1-10⵿

ESRD Ѵ.

31. MGN ݵǴ ȯ ๰

1) systemic disease

infection: HBV, HCV...

systemic autoimmune disease: SLE, RA, Sjogren's syndroeme, Hashimoto, Graves'...

neoplasia: breast, lung, colon, stomach, esophageal cancer..

2) drug: gold, penicillamine, captopril, NSAIDs...

ϱ: N PC MGN Ų.

32. MGNȯڰ ڱ ű ȭǾ

i) acute RVT

ii) superimposed CGN

iii) drug-induced interstitial nephritis

33. MGN poor prognostic factor

male, old age at onset, hypertension, Cr, severe hyperlipidemia, proteinuria>10 g/d

34. MGN poor prognostic factor on histology

advanced glomerular capillary wall alteration, segmental sclerosis, interstitial fibrosis,

tubular atrophy

35. Asymptomatic recurrent hematuria

IgA nephropaty, thin membrane disease

36. IgA nephropathy poor prognosis

older age at onset, gross hematuria(-), hypertension,

persistent & severe proteinuria, male, Cr

severe proliferation and sclerosis histology and/or tubulointerstitial change &

crescent formation

37. IgA nephropathy ȯ

HSP

liver - chronic liver disease

GI - Celiac ds, Crohn's disease, adenocarcinoma

lung - idiopathic interstitial pneumonitis, obstructive bronchiolitis, adenoca

skin - dermatitis heretiformis, mycosis fungoides, leprosy

38. IgA nephropathy ӽ ؾ ϴ

renal function(GFR<70 ml/min)

heavy or moderate proteinuria

ӽ

* ӽ

preserved renal function(GFR>70 ml/min)

mild proteinuria

minimal or absent hypertension

39. Thin BM disease(benign hematuria)

children <275 nm adult <300 nm

40. 24ð 輳Ǵ Creatinine =(28-0.2age)wt

ܹ鴢: 30-150 mg/d

dipstick test proteinura false positive: highly alkaline urine

41. Isolated proteinuria renal biopsy

most common : mild mesangial proliferative GN c/s FSGS(30-70%)

focal/diffuse proliferative GN(-15%)

interstitial nephritis(-5%)

20-40% 20 renal insufficiency ߻ ESRD 幰.

42. diabetic nephropathy ƴ ǽؾ ϴ

I) retinopathy

ii) 10 ̸ duration

iii) unusual UA: RBC cast

iv) overt nephropathy hypertension azotemia

v) unusually rapid deterioration of renal function

43. PAN Ȯ : ħ biopsyϿ vasculitis

biopsy Ұϴٸ angiography: small & medium-sized arery aneurysm

(renal & hepatic & visceral vasculature)

44. SLE Ab

anti-DNA(70%) high titer = nephritis & clinical activity

anti-smith Ab(30%) SLE specific

anti-RNP(40%) polymyositis, lupus, scleroderma, MCTD high titer

SLE anti-DNA anti-RNP ִٸ nephritis risk .

45. Lupus nephritis biopsy

chronicity = glomerulosclerosis, fibrous crescent, interstitial fibrosis, tubular atrophy

chronicity ġῡ ڴ.

46-1) HBV õ glomerulopathy

MGN, MPGN, IgA nephropathy, essential mixed cryoglobulinemia, polyarteritis nodosa

2) HIV : FSGS(mc), acute diffuse proliferative GN

3) HCV: proliferative GN, MPGN, MGN, cryoglobulinemia

47. multiple myeloma Ͼ ִ ȯ

myeloma kidney(cast nephropathy), amyloidosis, hypercalcemic nephropathy,

light chain nephropathy, uric acid nephropathy, Fanconi syndrome

dehydration(prerenal azotemia)

48. ̽ FSGS ġ? plasmapheresis, high dose cyclosporine

cf. ̽ FSGS ġ û ȯڸ ϱ ɼ ִ ?

plasmapheresis or protein A immunoadsorption

49. persistent isolated proteinuria 20-40% 20 renal insufficiency

ESRD 幰.

50. persisitent isolated proteinuria Ұ mild mesangial proliferative

glomerulonephritis̴.

51. MCD selective proteinuria(albumin ҽ) δ.

52. Lupus nephritis ġῡ monitorϰ, ϴµ

parameter Ͻÿ.

: activity of urine sediment, proteinuria, GFR, serum complement levels, anti-dsDNA titer

53. light chain ߻Ҽ ִ

amyloidosis, light-chain deposition disease, cast nephropathy