Glomerulonephritis Flashcards

1
Q

The presence of crescents correlates with the syndrome of _____ and carries a ______ prognosis.

A

RPGN; ominous

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2
Q

How do children present with Post-infectious Glomerulonephritis?

A

with edema and sudden weight gain

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2
Q

What lab findings are assoc. with Post-infectious Glomerulonephritis?

A

elevated levels of antibodies to streptococcal antigens (ASO titer, streptozyme) and decreased levels of complement components C3 with normal levels of C4 suggesting alternate complement pathway activation.

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2
Q

What is the tx for Post-infectious Glomerulonephritis?

A

none- it’s usually self limiting

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2
Q

What is the tx for auci-immune small vessel vasculitis?

A

immunosuppressive drugs (high-dose steroids and cyclophosphamide or rituximab)

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3
Q

Who gets MPGN?

A

older children and adolescents, esp females

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4
Q

What is the pattern of immune complex deposition in anti-GBM disease?

A

linear

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5
Q

______ is a type of GN that classically occurs after a _____ infection.

A

Acute post-infectious GN; Group-A strep

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5
Q

Who gets Goodpasture’s Syndrome?

A

young males

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5
Q

What is the pattern of immune complex deposition in lupus patients?

A

lumpy bumpy

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6
Q

______ can cause a severe, rapidly progressive GN, which may be accompanied by pulmonary hemorrhage (Goodpasture’s syndrome) or may be renal-limited.

A

Anti-GBM nephritis

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6
Q

What is Pauci-Immune Renal Vasculitis?

A

a small vessel vasculitis without immune complex deposition

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7
Q

This is glomerulonephritis from binding of antibody to an antigens in type IV collagen within the GBM.

A

Anti-GBM Disease

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8
Q

How do IgA nephropathy pts present?

A

asymptomatic microhematuria , nonnephrotic proteinuria, and normal or only mildly reduced renal function

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8
Q

What is Goodpasture’s syndrome?

A

the triad of pulmonary hemorrhage, iron deficiency anemia and GN associated with circulating antibody to GBM

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9
Q

How does look histologically?

A

fibrinoid necrosis and crescents without immune complex deposition

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10
Q

IgA nephropathy is the most common type of acute glomerulonephritis and usually presents in which people?

A

males 15 to 35 years old

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12
Q

Name 9 diseases with systemic symptoms that are associated with glomerulonephritis.

A
  • Henoch-Schönlein Purpura
  • Goodpasture’s syndrome
  • Lupus nephritis
  • ANCA associated vasculitis
  • Infection associated
  • Post-infectious
  • Endocarditis/shunt nephritis
  • Cryoglobulinemia
  • Immunoglobulin deposition diseases
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12
Q

MPGN refers to a histologic pattern in which there is both proliferation as well as thickening of the _____.

A

GBM

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13
Q

MPGN type I is most often idiopathic or associated with _____ infection.

A

HCV

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15
Q

The excretion of ____, _____, and _____ reflects glomerular inflammation and disruption of the GBM.

A

red cells, white cells and red cell casts

16
Q

How is Anti-GBM disease treated?

A

aggressive treatment with steroids, immunosuppressive agents and plasma exchange

18
Q

______ is the most common type of acute glomerulonephritis now seen worldwide and usually presents in males 15 to 35 years old.

A

IgA nephropathy

20
Q

Post-infectious Glomerulonephritis is diagnosed in patients presenting with nephritic syndrome with a positive ______ test and low ____ levels.

A

streptozyme; C3

21
Q

More than ____ of the patients with lupus develop clinically evident renal involvement.

A

half

22
Q

______ should be suspected in any patient with known hepatitis C infection who develops renal disease.

A

Cryoglobulinemic glomerulonephritis

24
Q

What is Anti-GBM Disease?

A

Glomerulonephritis from binding of antibody to an antigens in type IV collagen within the GBM

25
Q

This syndrome is the triad of pulmonary hemorrhage, iron deficiency anemia and GN associated with circulating antibody to GBM.

A

Goodpasture’s Syndrome

26
Q

In this disease, the renal lesion is similar to IgA nephropathy but more severe with a focal proliferative necrotizing glomerulonephritis, often with crescent formation, accompanied by mesangial and capillary wall deposits of IgA.

A

Henoch-Schonlein purpura (HSP)

28
Q

How does proteinuria occur?

A

direct damage to the glomerular capillary wall induced by immunologic mechanisms leads to an increase in protein filtration

29
Q

Which deposits form a characteristic “fingerprint” appearance?

A

cryoglobulinemia immune complexes

31
Q

Name 6 diseases, limited to the kidneys, that are associated with glomerulonephritis.

A
  • IgA nephropathy
  • Anti-GBM disease
  • Membranoproliferative glomerulonephritis (MPGN) 
  • Renal limited vasculitis (ANCA associated)
  • Hemolytic-uremic syndrome
  • C3 glomerulopathy/Dense deposit disease
33
Q

What is pulmonary-renal syndrome?

A

renal disease with pulmonary hemorrhage

34
Q

What is IgA nephropathy?

A

a form of mesangial proliferative glomerulonephritis in which there is a predominance of IgA immune deposits in the mesangium and rarely in subendothelial areas

35
Q

What are the characteristics of nephritic syndrome?

A
  • hematuria
  • dysmorphic red blood cells and/or red blood cell casts
  • proteinuria
35
Q

This disease is thought to be mediated by the deposition of IgA immune complexes to the mesangium with activation of mesangial cells via the Fc alpha receptors, resulting in cell proliferation and matrix expansion.

A

IgA nephropathy

36
Q

______ refers to a histologic pattern in which there is both proliferation as well as thickening of the GBM.

A

MPGN

37
Q

How does Anti-GBM disease present?

A

pulmonary hemorrhage with iron deficiency anemia

38
Q

Approximately 90% of patients with pauci-immune small vessel vasculitis have detectable ______.

A

anti-neutrophil cytoplasmic antibodies (ANCA)

39
Q

What is the tx for IgA nephropathy?

A

ACE inhibitors and possibly steroids with/without azathioprine

40
Q

Why does HTN occur in nephritic syndrome?

A

a consequence of salt and water retention

41
Q

What will the GFR be in nephritic syndrome?

A

it’s reduced

42
Q

This is a form of mesangial proliferative glomerulonephritis in which there is a predominance of IgA immune deposits in the mesangium and rarely in subendothelial areas.

A

IgA nephropathy

43
Q

What does RPGN stand for?

A

rapidly progressive glomerulonephritis

44
Q

Most patients with ______ symptomatic disease develop palpable purpura, arthralgias, and generalized weakness.

A

cryoglobulinemia

46
Q

In nephritic syndrome, what will the urine protein value be?

A

generally less than 3.0 gm/day

47
Q

Why does edema occur in nephritic syndrome?

A

an increase in tubular reabsorption of salt and water due to reduced, glomerular perfusion with well preserved tubular function leading to expansion of extracellular fluid volume

48
Q

The classic clinical manifestations of _____ in children include skin lesions (palpable purpura), arthritis, GI involvement (colic and bleeding) and glomerulonephritis.

A

Henoch-Schonlein purpura (HSP)

49
Q

This is a small vessel vasculitis without immune complex deposition.

A

Pauci-Immune Renal Vasculitis

50
Q

Glomerular disease should be considered in patients presenting with ____ and/or _____.

A

proteinuria; hematuria