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FA - Renal > Renal - Pathology (Nephrotic & Nephritic syndromes) > Flashcards

Renal - Pathology (Nephrotic & Nephritic syndromes) Flashcards

Pg. 536-538 in First Aid 2014 Pg. 490-492 in First Aid 2013 Sections include: -Nephrotic syndrome -Nephritic syndrome

1
Q

How does nephrotic syndrome present?

A

NephrOtic syndrome presents with (1) massive prOteinuria (>3.5g/day, frothy urine), (2) hyperlipidemia, (3) fatty casts, (4) edema

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2
Q

With what 2 other conditions is nephrotic syndrome associated, and why?

A

Associated with (1) thromboembolism (hypercoagulable state due to AT III loss in urine) and (2) increased risk of infection (loss of immunoglobulin)

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3
Q

What characterizes focal segmental glomerulosclerosis on light microscopy? What characterizes it on electron microscopy?

A

LM - Segmental sclerosis and hyalinosis; EM - Effacement of foot process similar to minimal change disease

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4
Q

What characterizes membranous nephropathy on light microscopy? What characterizes it on electron microscopy?

A

LM - Diffuse capillary and GBM thickening; EM - “spike and dome” appearance with subendothelial deposits

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5
Q

How does membranous nephropathy appear upon immunofluorescence? With what condition’s presentation is this immunofluorescence associated?

A

Granular; SLE’s nephrotic presentation

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6
Q

What characterizes minimal change disease on light microscopy? What characterizes it on electron microscopy?

A

LM - Normal glomeruli (lipid may be seen in PCT cells); EM - Foot process effacement

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7
Q

What characterizes amyloidosis on light microscopy?

A

LM - Congo red stain shows apple-green birefringence under polarized light

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8
Q

What characterizes diabetic glomerulonephropathy on light microscopy?

A

LM - mesangial expansion, GBM thickening, eosinophilic nodular glomerulosclerosis (Kimmelstiel-Wilson lesion).

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9
Q

What are the 6 glomerular diseases primarily classified as nephrotic syndrome?

A

(1) Focal segmental glomerulosclerosis (2) Membranous nephropathy (3) Minimal change disease (lipoid nephrosis) (4) Amyloidosis (5) Membrano-proliferative glomerulonephritis (MPGN) (Note: MPGN may be classified as both nephrotic and nephritic syndromes) (6) Diabetic glomerulonephropathy

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10
Q

What are the 5 glomerular diseases that are primarily classified as nephritic syndromes?

A

(1) Acute poststreptococcal glomerulonephritis (2) Rapidly progressive (crescentic) glomerulonephritis (RPGN) (3) Diffusive proliferative glomerulonephritis (DPGN) (Note: Diffusive proliferative glomerulonephritis may be classified as both nephrotic and nephritic syndrome) (4) Berger’s disease (IgA nephropathy) (5) Alport syndrome

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11
Q

What is the most common cause of nephrotic syndrome in adults? What is the second most common cause of primary nephrotic syndrome in adults?

A

Focal segmental glomerulosclerosis; Membranous nephropathy

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12
Q

What are 5 conditions/factors with which focal segmental glomerulosclerosis is associated?

A

Associated with (1) HIV infection, (2) Heroin abuse, (3) Massive obesity, (4) Interferon treatment, and (5) Chronic kidney disease due to congenital absence or surgical removal.

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13
Q

Again, what is the second most common cause of primary nephrotic syndrome in adults?

A

Membranous nephropathy

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14
Q

What factors can cause membranous nephropathy?

A

Can be idiopathic or caused by drugs (e.g., NSAIDs, penicillamine), infections (e.g., HBC, HCV), SLE, solid tumors.

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15
Q

What is loss in minimal change disease (lipid nephrosis), what is not loss, and why?

A

Selective loss of album, not globulins, caused by GBM polyanion loss

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16
Q

What might trigger minimal change disease (lipid nephrosis)?

A

May be triggered by a recent infection, immunization, or an immune stimulus.

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17
Q

In what patient population is minimal change disease (lipid nephrosis) most common?

A

Most common in children.

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18
Q

What is important to know about the response of minimal change disease (lipid nephrosis) to steroid therapy?

A

Excellent response to corticosteroids.

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19
Q

With what conditions is amyloidosis associated?

A

Associated with chronic conditions (e.g., multiple myeloma, TB, RA).

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20
Q

What are the types of membranoproliferative glomerulonephritis (MPGN)? What characteristics distinguish them?

A

TYPE I - subendotheial IC (immune complex) deposits with granular IF; “tram-track” appearance due to GBM splitting caused by mesangial in growth; TYPE II - intermembranous IC deposits; “dense deposits”

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21
Q

Which type of membranoproliferative glomerulonephritis (MPGN) is associated with “tram-track” appeaerance? What causes this appearance?

A

TYPE I - subendotheial IC deposits with granular IF; “tram-track” appearance due to GBM splitting

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22
Q

Which type of MPGN is associated with “dense deposits”?

A

TYPE II - intermembranous IC deposits; “dense deposits”

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23
Q

Among other types of nephrotic syndrome, what is unique about the presentation of MPGN?

A

(In addition to presenting as nephrotic syndrome) Can also present as nephritic syndrome

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24
Q

With what pathogens is Type I MPGN associated? What is another etiology to consider for Type 1 MPGN?

A

Type I is associated with HBV, HCV; May also be idiopathic

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25
Q

With what substance is Type II MPGN associated? What role does this substance play?

A

Type II is associated with C3 nephritic factor (stabilizes C3 convertase –> decrease serum C3 levels)

26
Q

What effects does nonenzymatic glycosylation (NEG) have in diabetic glomerulonephropathy?

A

(1) Nonenzymatic glycosylation (NEG) of GBM –> increased permeability, thickening; (2) NEG of efferent arterioles –> increased GFR –> mesangial expansion

27
Q

What characterizes nephritic syndrome and its presentation?

A

NephrItic syndrome = an Inflammatory process. When it involves glomeruli, it leads to hematuria and RBC casts in urine.

28
Q

With what signs/symptoms is nephritic syndrome associated?

A

Associated with azotemia, oliguira, hypertension (due to salt retention), and proteinuria (<3.5 g/day)

29
Q

What characterizes acute poststreptococcal glomerulonephritis on light microscopy? What characterizes it on electron microscopy?

A

LM - glomeruli enlarged and hypercellular; EM - subepithelial immune complex (IC) humps

30
Q

What characterizes acute poststreptococcal glomerulonephritis upon immunofluorescence, and what causes this?

A

IF - (“starry sky”) Granular appearance (“lumpy-bumpy”) due to IgG, IgM, and C3 deposition along GBM and mesangium

31
Q

In what patient population is acute poststreptococcal glomerulonephritis seen?

A

Most frequently seen in children

32
Q

What are 3 signs associated with acute poststreptococcal glomerulonephritis presentation?

A

(1) Peripheral and periorbital edema, (2) Dark urine (cola-colored), and (3) Hypertension

33
Q

How is acute poststreptococcal glomerulonephritis resolved?

A

Resolves spontaneously

34
Q

What characterizes rapidly progressive glomerulonephritis (RPGN) on light microscopy and electron microscopy?

A

LM and IF - crescent-moon shape

35
Q

In what glomerular disease are crescents found? What do crescents consist of?

A

Rapidly progressive (crescentic) glomerulonephritis (RPGN); Crescents consist of fibrin and plasma proteins (e.g., C3b) with gomerular parietal cells, monocytes, and macrophages

36
Q

What is the prognosis associated with RPGN, and why?

A

Poor prognosis. Rapidly deteriorating renal functions (days to weeks)

37
Q

What are 3 disease processes that may result in the pattern of RPGN?

A

(1) Goodpasture’s syndrome (2) Granulomatosis with polyangitis (Wegener’s) (3) Microscopic polyangitis

38
Q

What kind of hypersensitivity is Goodpasture’s syndrome?

A

Type II hypersensitivity

39
Q

What kind of immunofluorescence is seen in Goodpasture’s syndrome, and what causes this?

A

Antibodies to GBM and alveolar basement membrane –> linear IF

40
Q

Again, what are the 3 disease processes that may result in the pattern of RPGN? What is used to make a diagnosis in each case?

A

(1) Goodpasture’s syndrome - Hematuria/hemoptysis (2) Granulomatosis with polyangitis (Wegener’s) - PR3-ANCA/c-ANCA (3) Microscopy polyangitis - MPO-ANCA/p-ANCA

41
Q

What 2 things cause diffuse proliferative glomerulonephritis (DPGN)?

A

Due to (1) SLE or (2) MPGN.

42
Q

What characterizes diffuse proliferative glomerulonephritis (DPGN) on light and electron microscopy? What characterizes it upon immunofluorescence?

A

LM - “wire looping” of capillaries; EM - subendothelial and sometimes intramembranous IgG-based ICs often with C3 deposition; IF - granular

43
Q

What is the most common cause of death in SLE?

A

Diffuse proliferative glomerulonephritis (DPGN)

44
Q

What is unique about the presentation of DPGN and MPGN?

A

DPGN and MPGN can present as nephrotic syndrome and nephritic syndrome concurrently.

45
Q

To what other condition is Berger’s disease (IgA nephropathy) associated?

A

Related to Henoch-Schonlein purpura

46
Q

What kind of nephropathy is Berger’s disease?

A

IgA nephropathy

47
Q

What characterizes Berger’s disease on light microscopy, electron microscopy, and immunofluorescence?

A

LM - mesangial proliferation; EM - mesangial IC deposits; IF - Ig-A based IC deposits in mesangium

48
Q

What 2 conditions often causes Berger’s disease to present/flare? What clinical/lab findings are associated with Berger’s disease?

A

Often presents/flares with a URI or acute gastroenteritis; Episodic hematuria with RBC casts.

49
Q

What mutation causes Alport syndrome? What is the effect of this mutation?

A

Mutation in type IV collagen –> thinning and splitting of the glomerular basement membrane.

50
Q

What mode of inheritance is associated with Alport syndrome?

A

Most commonly X-linked

51
Q

What are 3 symptoms associated with Alport syndrome? Which of these is less common?

A

(1) Glomerulonephritis, (2) Deafness, and, less common, (3) Eye problems

52
Q

What is the most common cause of nephrotic syndrome in African Americans and Hispanics?

A

Focal segmental glomerulosclerosis is the most common cause of nephrotic syndrome in African Americans and Hispanics

53
Q

What is important to know about the response of FSGS to steroid therapy?

A

Inconsistent response to steroid therapy and may progress to chronic renal disease

54
Q

What is the most common cause of primary nephrotic syndrome in Caucasian adults?

A

Membranous nephropathy is the most common cause of primary nephrotic syndrome in Caucasian adults

55
Q

What is important to know about the response of membranous nephropathy to steroid therapy?

A

Poor response to steroid therapy and may progress to chronic renal disease.

56
Q

With what condition may minimal change disease (lipoid nephrosis) be associated?

A

May be associated with Hodgkin lymphoma (e.g., cytokine-mediated damage).

57
Q

What is the organ most commonly involved in amyloidosis?

A

Kidney is the most commonly involved organ (systemic amyloidosis).

58
Q

When does acute poststreptococcal glomerulonephritis typically occur?

A

Occurs ~ 2 weeks after group A streptococcal infection of the pharynx or skin.

59
Q

What type of hypersensitivity reaction is acute poststreptococcal glomerulonephritis?

A

Type III hypersensitivity reaction.

60
Q

What main 2 lab values are associated with acute poststreptococcal glomerulonephritis?

A

(1) Increased anti-DNase B titers and (2) Decreased complement levels

61
Q

Which nephritis would you consider in a patient with episodic hematuria with RBC casts?

A

IgA nephropathy (Berger disease)

FA - Renal (13 decks)

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