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Ch 31 Primary GN > FSGS > Flashcards

FSGS Flashcards

(42 cards)

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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?

18
Q

IF findings in FSGS?

Study These Flashcards
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?

Study These Flashcards
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?

Study These Flashcards
A

NPHS2 (gene for podocin)

21
Q

What is the gene encoding for Podocin?

22
Q

What is the gene encoding for Nephrin?

23
Q

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

Study These Flashcards
A

NPHS1 (gene for Nephrin)

24
Q

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

25
Mutations in this gene are a cause of familial FSGS, with an autosomal dominant pattern of inheritance?
ACTN4
26
What gene in Africans is associated with kidney disease?
APOL1
27
What distinguishes HIV-associated FSGS from idiopathic collapsing FSGS?
PRESENCE OF ENDOTHELIAL TUBULORETICULAR INCLUSIONS
28
What virus has been associated with idiopathic and collapsing FSGS?
PATVOVIRUS B19
29
What are the 3 medications which causes Collapsing FSGS?
1. PAMIDRONATE 2. INTERFERON 3. ANABOLIC STEROIDS
30
What is the hallmark feature of all forms of primary FSGS?
PROTEINURIA
31
What is the more common presentation of FSGS in adults?
HYPERTENSION
32
What FSGS variant is more common among older Caucasian males?
TIP
33
What FSGS variant is more likely to attain complete remission?
TIP
34
What FSGS variant is associated with better renal survival?
TIP
35
In FSGS, what correlates with long-term renal survival?
ENTRY SERUM CREATININE LEVEL
36
What entry creatinine level in FSGS portends a poorer renal survival?
> 1.3mg/dl
37
What are the 3 Drugs used in the treatment of FSGS?
1. ACE INHIBITORS (1st step: BP control) 2. GLUCOCORTICOIDS (for nephrotic range proteinuria) 3. CYCLOSPORINE (if with CI to corticosteroids or steroid-resistant)
38
What is the median time for complete remission after prednisone therapy in FSGS?
3-4 months
39
What drug is used for steroid-resistant FSGS?
CYCLOSPORINE
40
How long should patients with FSGS be treated with cyclosporine?
12 months
41
What are the 2 side effects of long term use of Cyclosporine?
1. Interstitial fibrosis 2. Tubular atrophy
42
What is the dose of cyclosporine that is associated with increased side effects?
> 5.5 mg/kg/day

Ch 31 Primary GN (10 decks)

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