Glomerulonephritis Flashcards Preview

CVPR: Renal > Glomerulonephritis > Flashcards

Flashcards in Glomerulonephritis Deck (28)
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1

Nepritic syndromes characteristics/signs

  • classic presentation of pts w/glomerulonephritis
  • reduction of GFR ==> elevated serum creatinine
  • hematuria + dysmorphic RBCs and/or RBC casts + proteinuria
    • WBCs also possible
  • hypertension + edema

2

Typical renal-limited diseases associated w/glomerulonephritis

  • IgA nephropathy
  • Anti-GBM disease
  • Membranoproliferative glomerulonephritis (MPGN)
  • Renal limited vasculitis (ANCA associated)
  • Hemolytic-uremic syndrome
  • C3 glomerulopathy/Dense deposit disease

3

Typical systemic diseases associated w/glomerulonephritis

  • Henoch-Schönlein Purpura
  • Goodpasture’s syndrome
  • Lupus nephritis
  • ANCA associated vasculitis
  • Infection associated
  • Post-infectious
  • Endocarditis/shunt nephritis
  • Cryoglobulinemia
  • Immunoglobulin deposition diseases

4

Common labs/serology evaluation in glomerulonephritis

  • lab: CBC, electrolyte, 24-hour urine, liver fxn tests
  • serology:
    • C3 (complement) levels
    • antistreptolysin (ASO) titer
    • ANA, ANCA, cryoglobulins, anti-GBM

5

Pulmonary-renal syndrome definition

  • patients presenting w/glomerulonephritis + pulmonary hemorrhage
  • e.g. in Goodpasture's or ANCA-associated vasculitis

6

Blood tests + disease associations in glomerulonephritis

ANA, Anti-DS DNA

Lupus

C3, C4

See table

Blood cultures

Bacterial endocarditis

ASO

Post-infectious

Hepatitis C

Cryoglobulinemia

Hepatitis B

Membranoproliferative glomerulonephritis

Anti-neutrophil cytoplasmic antibodies (ANCA)

Pauci-immune vasculitis

Anti-GBM antibodies

Anti-GBM disease, Goodpasture’s syndrome

7

Pathophysiology of proteinuria in glomerulonephritis

  • direct damage to glomerular capillary wall via immunologic mechanisms ==> increased protein filtration

8

Pathophysiology of reduction of GFR in glomerulonephritis

  • reduced GFR <== acute, inflammatory process w/in glomerulus
  • ==> glomerular vasocontsriction/occlusion or thrombosis ==> decreased filtration surface area

9

Pathophysiology of active urine sediment in glomerulonephritis

  • excretion of RBCs, WBCs, or casts <== glomerular inflammation + disruption of GBM

10

Pathophysiology of edema/hypertension in glomerulonephritis

  • edema <== increase in tubular reabsorption of salt + water <== reduced glomerular perfusion w/normal tubular fxn ==> expansion of ECF volume
  • HTN <== salt + water retention

11

Epidemiology/etiology: Post-infectious glomerulonephritis

  • occurs 2-3 wks after Group A strep infection
  • most common in children
  • may occur w/acute or subacute active infections
  • mediated by Ab response to strep ==> immune complex deposition + activation of complent @ glomeruli

12

Pathology/Clinical Presentation: Post-infectious glomerulonephritis

  • light microscope: proliferative + exudative w/inflitration of PMNs and monocytes
  • Immunofluorescence: IgG + C3 deposits @ subendothelial, mesangial, subepithelial
  • clinical present:
    • edema + sudden weight gain + HTN
    • hematuria + proteinuria
    • decreased GFR
    • Positive ASO titer
    • decreased C3 w/normal C4

13

Diagnosis/Tx: Post-infectious glomerulonephritis

  • Dx: nephritic syndrome + positive streptozyme + low C3
  • Tx: supportive

14

Epidemiology/etiology: IgA Nephropathy

  • most common; usually males 15-35
  • mediated by deposition of IgA complexes @ mesangium ==> activation of mesangial cells ==> cell proliferation

15

Pathology/Clinical: IgA Nephropathy

  • light: increase in mesangial cell number/matrix
  • IF: IgA, IgG, C3 @ mesangium
  • Clinical:
    • usually asymptomatic
    • often increased hematuria w/onset of viral illness
    • systemic sx possible
    • skin w/IgA deposits = Henoch-Schonlein purpura

16

Epidemiology/etiology: MPGN

  • MPGN = mesangial proliferation + thickeneing of GBM
  • epidemiology = associated w/systemic illness or idiopathic<== adolescents + female > male

17

Pathology/Pathogenesis: MPGN

  • LM: thick basement membrane, mesangial cell proliferation + lobulated appearance
    • Deposits @ capillary walls, mesangium: C3, IgG
  • EM: subendothelial + mesangial deposits
  • Pathogenesis: trapping of circulating immune complexes

18

Clinical Presentation: MPGN

  • acute glomerulonephritis or nephritic syndrome
  • early HTN
  • common test results: Hep C infection w/HCV and RNA in blood + cryoglobulins
    • RF
    • low complement (C3 & C4)

19

Tx/Prognosis: MPGN

  • poor prognosis ==> end-stage renal disease
  • sometimes steroids
  • Hep C + ==> anti-viral

20

Pathology: Anti-GBM disease

  • Ab binds to type IV collagen w/in GBM ==> linear IgG deposits ==> glomerulonephritis
  • Abs ==> complement activation + PMN infiltration
  • crescent formation + rapid loss of renal fxn

21

Clinical Presentation/Tx/Prognosis: Anti-GBM disease

  • pulmonary hemorrhage + iron deficiency anemia + nephritis
  • Tx: steroids + immunosuppresion + plasma exchange ==> increases renal survival

22

Pathology/Pathogenesis: Pauci-Immune Renal Vasculitis

  • PIRV=small vessel vasculitis involving the kidney but w/out immune-complex deposition
  • Path: fibrinoid necrosis + crescents
  • Pathogen: 
    • ANCA (anti-neutrophil cytoplasmic Ab) positive

23

Clinical Presentation/Tx:  Pauci-Immune Renal Vasculitis

  • Can present @ skin, lungs, GI (and renal)
    • w/lungs = pulmonary-renal syndrome
  • nephritic pattern of renal disease
  • Tx: immunosuppresive drugs; steroids + cyclophosphamide or rituximab
    • possible: plasma exchange; dialysis

24

Pathology/Pathogenesis: Lupus Nephritis

  • Path: immune-complexes @ mesangium, subepithelium, subendothelium w/lumpy-bumpy pattern
  • Pathogen: autoantibody disease e.g. ANA (Anti-nuclear antibody)

25

Clinical Presentation/Tx: : Lupus Nephritis

  • Renal signs: heamturia, proteinuria
    • either nephritic or nephrotic
  • Tx: steroids + cyclophosphamide

26

Clinical Presentation/Tx: Cryoglobulinemia

  • Systemic sx: palpable purpura, arthralgias, generalized weakness
  • Urine: Proteinuria, hematuria
  • Labs: low C4, cryoglobulins, RF
  • Tx: antiviral (w/Hep C)
    • rituximab if lymphoproliferative disease
    • plasmapheresis

27

Pathology/Pathogenesis: Cryoglobulinemia

  • Path: immune-complex glmerulonephritis
    • memranoproliferative pattern of injury + subendothelial immune deposition
  • Pathogen: Cryoglobulins = frequently associated w/Hep C infection
    • cooling blood ==> immune complex formation

28

Characteristics of Henoch-Schonlein purpura (HSP)

  • clinical:
    • skin lesions (palpable purpura)
    • arthritis
    • GI involvement
    • glomerulonephritis
  • path:
    • focal proliferative necrotizing glomerulonephritis w/crescent formation
    • mesangial + cap wall deposits of IgA