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*MHD Exam 5 > Nephritic Diseases > Flashcards

Nephritic Diseases Flashcards

1
Q

Is the onset of nephritic syndrome usually acute or insidious?

A

acute?

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2
Q

What are the key findings associated with nephritic syndrome?

A
  • hematuria w/ dysmorphic cells and RBCs/casts in urine
  • some oliguria and azotemia
  • HTN

(may also see edema and mild proteinuria)

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3
Q

What is the typical clinical presentation of acute post-infectious glomerulonephritis in adults vs. children?

A
  • adults: acute nephritic syndrome uncommon

- children: acute nephritic syndrome w/ hematuria, edema, HTN, and renal failure

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4
Q

Epidemiology of post-infectious glomerulonephritis?

A
  • children 6-10 y/o
  • sporadic or epidemic
  • rarer in adults
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5
Q

What is the etiology/pathogenesis of post-infectious glomerulonephritis?

A
  • 1-4 weeks after recovery from infection
  • usually group A beta-hemolytic strep infection, although pneumococcal, staphylococcal, and viral are possible
  • Abs against M protein leads to immune complex formation
  • Immune complexes deposit in glomeruli -> activates complement -> attracts PMNs -> mediate damage w/ endocapillary proliferation, structural damage, hematuria
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6
Q

Post-infectious glomerulonephritis is a prototypic disease of:

A

circulating immune complex glomerulonephritis

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7
Q

In post-infectious glomerulonephritis, what are Abs directed against?

A

the infectious agent and NOT glomerular components

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8
Q

unique to post-infectious glomerulonephritis and therefore diagnostically useful

A

in-situ immune complexes leading to formation of large subepithelial deposits (“humps”)

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9
Q

Where do immune complexes deposit in post-infectious glomerulonephritis?

A

in subendothelial space (proximal zone) AND subepithelial space (distal zone)

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10
Q

What would you see on immunofluorescence with post-infectious glomerulonephritis?

A

glomerulus with immune complex deposits of IgG+complement (bright green spots)

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11
Q

Which lab tests are helpful in the diagnosis of post-infectious glomerulonephritis?

A
  • tea-colored (smoky, Coca Cola colored) urine
  • hematuria and mild proteinuria
  • incr. ASO titer
  • decr. complement
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12
Q

Prognosis of post-infectious glomerulonephritis?

A
  • children: total recovery w/ resolution of pathology 95% of time
  • adults: slow progression to chronic glomerulonephritis w/ 15-50% developing ESRD
  • small subset of children and adults may develop very severe acute illness w/ rapidly progressive renal failure
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13
Q

IgA is the major immunoglobulin in _______ and second most common immunoglobulin in _______.

A

secretions/mucosa; serum

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14
Q

How does IgA affect the complement system?

A

It is a poor activator, and complement involvement is solely via alternative complement pathway.

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15
Q

In IgA nephropathy, immune complexes contain:

A

IgA + C3 complement

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16
Q

Which isotype of IgA is more common in secretions?

A

IgA2 (IgA1 predominates in serum)

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17
Q

also known as Berger disease

A

IgA nephropathy

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18
Q

What is the typical clinical presentation of IgA nephropathy?

A
  • RECURRENT gross and microscopic hematuria
  • episodes of gross hematuria within 1-2 days of nonspecific URI (also GI or UTI)
  • painless hematuria following infection
  • hematuria for days, recurrence every few months
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19
Q

What is the pattern of hematuria associated with IgA nephropathy?

A
  • recurrent gross and microscopic hematuria

- painless, gross hematuria within 1-2 days of infection

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20
Q

What is the most common glomerular disease worldwide?

A

IgA nephropathy

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21
Q

IgA nephropathy is most common in which patient population?

A

children and young adults

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22
Q

What plays a central role in the etiology/pathogenesis of IgA nephropathy?

A

abnormally glycosylated IgA1

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23
Q

What is the role of genetics in IgA nephropathy?

A

Geographic and racial differences have been recognized, and it is now clear that a large portion of the disease risk is conferred genetically. There have been at least 7 susceptibility loci for IgA nephropathy identified. These genes are associated with innate and adaptive immunity, as well as the complement system.

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24
Q

Where do IgA complexes deposit in IgA nephropathy?

A

in the mesangium

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25
Q

Celiac disease has been associated with which of the nephropathies?

A

IgA nephropathy (due to abnormality of immune regulation)

26
Q

What are the 4 hits in the multi-hit pathogenesis model of IgA nephropathy?

A
  • 1: incr. production of galactose-deficient IgA1 after respiratory/GI exposure to viruses, bacteria, food products
  • 2: formation of auto Abs that recognize abnormal IgA1
  • 3: formation of pathogenic immune complexes
  • 4: deposition of immune complexes in mesangium -> activation of mesangial cells -> induction of glomerular injury (via complement activation)
27
Q

How is IgA nephropathy diagnosed definitively?

A

IgA-containing immune complexes in mesangium on immunofluorescence assay

28
Q

What do lab tests show with IgA nephropathy?

A
  • heamturia
  • mild proteinuria
  • normal complement levels (replenished by liver)
29
Q

Prognosis for IgA nephropathy?

A

variable depending on glomerular pathology (many relatively indolent/prolonged, but may lead to renal failure; rare patients w/ rapidly progressive glomerulonephritis; systemic IgA vasculitis may occur in kids)

30
Q

IgA nephropathy with extra-renal symptoms is known as:

A

systemic IgA vasculitis, or Henoch-Shonlein purpura (HSP)

31
Q

What is the best-studied hereditary nephritis?

A

Alport syndrome (X-linked)

32
Q

What is the triad associated with Alport syndrome?

A
  • nephritis (isolated hematuria)
  • nerve deafness
  • various eye disorders (ex: early cataracts)
33
Q

Common cause of isolated hematuria?

A

hereditary nephritis

34
Q

Hereditary nephritis diseases involve what kind of mutations?

A

mutations leading to error in synthesis of collagen type IV, which is the major component of the GBM, cochlea, lens

35
Q

What is the difference b/t males and females in regard to clinical presentation of hereditary nephritis?

A
  • males: full spectrum of disease

- females: carriers, rarely with disease

36
Q

Epidemiology of hereditary nephritis?

A
  • age 5-20 at presentation

- 20-50 years w/ overt renal failure

37
Q

What is a critical part of the diagnosis of hereditary nephritis and why?

A

lamina densa splitting/lamination (“basket weave”) on electron microscopy; paraffin sections and immunofluorescence are non-diagnostic

38
Q

What are the lab tests to check for hereditary nephritis?

A
  • hematuria

- genetic testing

39
Q

Prognosis and treatment of hereditary nephritis?

A

overt renal failure b/t 20-50 years of age; treatment is mainly supportive, including counseling; may opt for a transplant; family testing recommended

40
Q

What is rapidly progressing glomerulonephritis (RPGN)?

A

A clinical syndrome consisting of:

  • rapidly progressive loss of renal function
  • nephritic syndrome w/ gross hematuria
  • crescents on histology
  • diverse pathogenesis
41
Q

What is hematuria a consequence of?

A

severe GBM injury (necrosis, breaks)

42
Q

What do crescents form in response to in RPGN?

A

hematuria; leakage of blood and fibrin into Bowman’s space leads to proliferation of parietal epithelial cells; eventually get a glomerular crescent w/ blood, fibrin, macs, and parietal epithelial cells

43
Q

What are the different types of RPGN?

A
  • Type I: anti-glomerular BM antibody disease
  • Type II: severe immune complex glomerulonephritis
  • Type III: “pauci-immune”/ANCA-associated
44
Q

Epidemiology of Type I RPGN?

A

young men (rare, 12% of crescentic glomerulonephritis cases)

45
Q

Etiology of RPGN Type I?

A

anti-glomerular BM antibodies that form as a result of exposure to toxins (viruses, smoking, solvents, drugs); patients also likely have a genetic predisposition to autoimmunity

46
Q

Which syndrome is RPGN Type I also known as?

A

Goodpasture

47
Q

What is the typical clinical presentation of RPGN Type I?

A
  • gross hematuria
  • drop in urine output (acute renal failure)
  • hemoptysis
48
Q

Why does Goodpasture result in pulmonary hemorrhage?

A

anti-glomerular BM antibodies cross-react with pulmonary alveolar basement membrane, resulting in hemoptysis

49
Q

What is seen on immunofluorescence with RPGN Type I?

A

linear IgG deposits (in glomeruli AND pulmonary alveoli)

50
Q

Treatment of Goodpasture?

A

plasmapheresis (removal of antigenic antibodies from circulation)

51
Q

What are the typical lab tests for a patient with RPGN Type I?

A
  • UA: abundant RBCs w/ casts
  • CBC: markedly elevated serum creatinine
  • CXR: bilateral pulmonary opacities
52
Q

What is the typical patient population that develops RPGN Type II?

A
  • older children and young adults (10-40 y/o)

- patients w/ SLE

53
Q

a severe variant of the prototype immune complex disease

A

RPGN Type II

54
Q

What is seen on immunofluorescence and EM with RPGN Type II?

A
  • ABUNDANT immune complexes (IgG+C3, IgA+C3) on immunofluorescence (“Christmas tree”)
  • electron dense deposits on EM
55
Q

What are potential etiologies of RPGN Type II?

A

SLE, IgA nephropathy, post-infectious glomerulonephritis

56
Q

Treatment of RPGN Type II?

A

immunosuppression

57
Q

What is the typical patient population affected by RPGN Type III?

A

older patients (>80% over 65 y/o)

58
Q

What is the typical clinical presentation of RPGN Type III?

A
  • drop in urinary output (acute renal failure)
  • gross hematuria
  • frequent (75%) extrarenal manifestations (hemoptysis, dyspnea)

*appears very similar to type I

59
Q

Main etiology of RPGN Type III?

A

ANCAs

60
Q

direct cause of pauci-immune crescentic glomerulonephritis/systemic small vessel vasculitis

A

ANCA-associated disease (RPGN Type III)

61
Q

What is seen on histology, immunofluorescence, and EM with RPGN Type III?

A

crescents on histology; negative IF and EM

62
Q

What are the ANCA vasculitides?

A
  • Granulomatosis w/ Polyangiitis (GPA)
  • Eosinophilic GPA (EGPA)
  • Microscopic polyangiitis (MPA)

*MHD Exam 5 (14 decks)

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