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Ch 31 Primary GN > FSGS > Flashcards

FSGS Flashcards

1
Q

What are the 5 major structural variants of FSGS?

A
  1. PERIHILAR
  2. GLOMERULAR TIP
  3. COLLAPSING
  4. CELLULAR
  5. NOS
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2
Q

What is the variant of FSGS that is clinically aggressive?

A

COLLAPSING VARIANT

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3
Q

What variant of FSGS has a good outcome?

A

GLOMERULAR TIP VARIANT

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4
Q

Variant of FSGS where foam cells are within consolidated segments

A

CELLULAR VARIANT

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5
Q

Variant of FSGS that is characterized by sclerosis at the hilum of the glomerulus that contains foci of hyalinosis

A

PERIHILAR VARIANT

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6
Q

What variant of FSGS that has a characteristic feature of focal segmental or global collapse of glomerular capillaries, with obliteration of capillary lumens?

A

COLLAPSING

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7
Q

What variant of FSGS is the major pathologic expression of HIV nephropathy?

A

COLLAPSING

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8
Q

What are the 3 conditions which exhibit Collapsing FSGS?

A
  1. HIV NEPHROPATHY
  2. IV DRUG ABUSE
  3. IDIOPATHIC
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9
Q

In a specimen with the collapsing glomerulopathy variant of FSGS, an important ultrastructural assessment is for the presence or absence of what?

A

ENDOTHELIAL TUBULORETICULAR INCLUSIONS

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10
Q

Endothelial Tubuloreticular lesions are found in how many percent in HIV-associated collapsing GN?

A

90%

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11
Q

Endothelial Tubuloreticular lesions are found in how many percent in idiopathic collapsing GN?

A

10%

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12
Q

What variant of FSGS that is characterized by consolidation of the glomerular segment that is adjacent to the origin of the proximal tubule and opposite the hilum?

A

GLOMERULAR TIP

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13
Q

What is the classification system for FSGS?

A

COLUMBIA CLASSIFICATION SYSTEM

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14
Q

What variant of FSGS resemble the cellular lesion for the tip variant, but they are distributed more widely in the glomerular tuft and are not confined to the tip

A

CELLULAR

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15
Q

What FSGS variant is characterized by the presence of hyalinosis?

A

PERIHILAR

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16
Q

What are the 2 causes of secondary FSGS that has a perihilar pattern and is accompanied by glomerular enlargement?

A
  1. OBESITY
  2. REDUCED NUMBER OF NEPHRONS
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17
Q

Foot process effacement affects both sclerotic and nonsclerotic glomeruli and usually is more focal in which type of GN?

A

FSGS

18
Q

IF findings in FSGS?

A

IgM (low level staining)
C3 (less frequent)
IgG, IgA (rare)

19
Q

What are the 5 genetic mutations linked to familial and sporadic cases of FSGS?

A
  1. (NPHS1): NEPHRIN
  2. (NPHS2): PODOCIN
  3. (ACTN4): α-actinin-IV
  4. (TRPC6): transient receptor potential cation channel, subfamily C, member 6
  5. (PLCE1): phospholipase Cε1
20
Q

What gene mutations are associated with familial & childhood-onset, steroid-resistant nephrotic syndrome?

A

NPHS2 (gene for podocin)

21
Q

What is the gene encoding for Podocin?

A

NPHS2

22
Q

What is the gene encoding for Nephrin?

A

NHS1

23
Q

What gene mutations are associated with congenital nephrotic syndrome of the Finnish type presenting within the first 3 months of life?

A

NPHS1 (gene for Nephrin)

24
Q

What gene encodes for the actin-binding protein α-actinin-4?

A

ACTN4

25
Q

Mutations in this gene are a cause of familial FSGS, with an autosomal dominant pattern of inheritance?

A

ACTN4

26
Q

What gene in Africans is associated with kidney disease?

A

APOL1

27
Q

What distinguishes HIV-associated FSGS from idiopathic collapsing FSGS?

A

PRESENCE OF ENDOTHELIAL TUBULORETICULAR INCLUSIONS

28
Q

What virus has been associated with idiopathic and collapsing FSGS?

A

PATVOVIRUS B19

29
Q

What are the 3 medications which causes Collapsing FSGS?

A
  1. PAMIDRONATE
  2. INTERFERON
  3. ANABOLIC STEROIDS
30
Q

What is the hallmark feature of all forms of primary FSGS?

A

PROTEINURIA

31
Q

What is the more common presentation of FSGS in adults?

A

HYPERTENSION

32
Q

What FSGS variant is more common among older Caucasian males?

A

TIP

33
Q

What FSGS variant is more likely to attain complete remission?

A

TIP

34
Q

What FSGS variant is associated with better renal survival?

A

TIP

35
Q

In FSGS, what correlates with long-term renal survival?

A

ENTRY SERUM CREATININE LEVEL

36
Q

What entry creatinine level in FSGS portends a poorer renal survival?

A

> 1.3mg/dl

37
Q

What are the 3 Drugs used in the treatment of FSGS?

A
  1. ACE INHIBITORS (1st step: BP control)
  2. GLUCOCORTICOIDS (for nephrotic range proteinuria)
  3. CYCLOSPORINE (if with CI to corticosteroids or steroid-resistant)
38
Q

What is the median time for complete remission after prednisone therapy in FSGS?

A

3-4 months

39
Q

What drug is used for steroid-resistant FSGS?

A

CYCLOSPORINE

40
Q

How long should patients with FSGS be treated with cyclosporine?

A

12 months

41
Q

What are the 2 side effects of long term use of Cyclosporine?

A
  1. Interstitial fibrosis
  2. Tubular atrophy
42
Q

What is the dose of cyclosporine that is associated with increased side effects?

A

> 5.5 mg/kg/day

Ch 31 Primary GN (10 decks)

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