ASN QBank Pearls - Glomerular and Vascular Disorders Flashcards

1
Q

have often been associated with increased risk for progression of diabetic
nephropathy

A

smoking and HTN

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2
Q

which genetic screening test is likely to detect a mutation in more than 5% of sporadic FSGS in adolescents?

A

mutations in NPHS2 (podocin) gene, including p.R229Q variant

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3
Q

possible kidney biopsy findings in MM nephropathy

A
  • fractured tubular casts
  • Congo red–positive glomerular and tubular deposits
  • acute tubular injury/necrosis
  • plasma cell infiltrates
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4
Q

amyloidosis fibril size

A

9 to 12 nm

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5
Q

cryoglobulinemic GN microtubule shape and size

A
  • curved

- 25-35 nm

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6
Q

immunotactoid glomerulopathy microtubule pattern and size

A
  • parallel arrays

- > 30 nm

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7
Q

fibrillary GN fibril pattern and size

A
  • RANDOM

- 15-30 nm

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8
Q

lupus nephritis IG pattern

A

“fingerprint”

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9
Q

treatment for idiopathic MCD

A
  • sodium and fluid restriction
  • furosemide
  • statins
  • ACEI
  • high-dose prednisone daily or on alternate days
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10
Q

spontaneous remission rate of nephrotic syndrome in iMCD

A

1/3, but may take up to 2 years or more

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11
Q

treatment of steroid-resistant FSGS

A

cyclosporine A often in combination with low-dose prednisone

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12
Q

lesion associated with highest response rate to treatment with steroids and 
has the BEST prognosis in nephrotic patients with FSGS

A

tip lesion

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13
Q

has not been shown to be effective treatment in patients with idiopathic membranous glomerulonephritis (iMGN)

A

prednisone alone

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14
Q

multiple myeloma with acute cast nephropathy clues

A
  • very low anion gap
  • hypercalcemia
  • anemia
  • presence of trace urine protein by dipstick
  • elevated UPC on direct measurement
  • protein electrophoresis shows IgG kappa paraprotein
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15
Q

HIVAN

A

collapsing FSGS

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16
Q

HIVAN bp

A

rarely hypertensive

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17
Q

hepatitis B–induced membranous GN is usually associated with

A

hepatitis BeAg immune complexes in the subEPIthelial space

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18
Q

hepatitis BsAg-induced MPGN is usually associated with

A

subENDOthelial deposits due to the different sizes of molecules

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19
Q

hepatitis C leads to what type of cryoglobulinemia

A

type 2

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20
Q

hepatitis C leads to what type of MPGN

A

type 1 MPGN

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21
Q

cryoglobulins activate which complement pathway

A

classical complement pathway

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22
Q

HIV directly infects renal tissue including the

A
  • podocytes
  • mesangial cells, and
  • renal tubular cells
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23
Q

associated renal lesion;

- hepatitis A

A

postinfectious GN (PIGN)

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24
Q

associated renal lesion;

- HIV

A

collapsing FSGS

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25
Q

associated renal lesion;

- hepatitis C

A

MPGN

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26
Q

associated renal lesion;

- hepatitis B

A

membranous GN

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27
Q

HIVAN or collapsing FSGS is due to

A

active viral replication in the podocyte –> podocytes change to proliferative macrophages

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28
Q

treatment that may lead to an improvement in renal function and nephrotic syndrome in HIVAN

A
  • HAART
  • ACEI or ARB, and
  • steroids
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29
Q

HIVAN predominantly diagnosed in patients of what race

A

black

30
Q

HIVAN predominantly affects what sex

A

males > females

31
Q

HIVAN RUS findings

A

echogenic, occasionally large-sized kidneys

32
Q

treatment for crescentic type I membranoproliferative glomerulonephritis (MPGN) secondary to cryoglobulinemia with no evidence of chronicity

A
  • initiation of HCV antiviral therapy 

  • plasmapheresis 

  • corticosteroids, and
  • rituximab
33
Q

NSAID use, AKI, and nephrotic syndrome

A

AIN and MCD

34
Q

cause of leukocytoclastic skin lesions (can be on legs and ears), AKI, small vessel vasculitis

A

cocaine use

35
Q
  • leukocytoclastic rash
  • nephritis
  • and abnormal liver function tests
  • undetectable C4
  • low to normal C3
A

cryoglobulinemia

36
Q

timeline of hematuria from PIGN

A

2 weeks after onset of infection

37
Q

sacroiliitis is associated with

A

human leukocyte antibody B27 (HLAB27) and IgA nephropathy

38
Q

associated with a high risk of relapse in pauci-immune necrotizing and crescentic glomerulonephritis (MPO-ANCA)

A

presence of upper respiratory tract disease (such as nasal crusting)

39
Q

MOST likely to be decreased in MPGN, especially C3 glomerulopathy

A

factor H (normal inhibitor of the alternative complement pathway)

40
Q

SLE and TMA, think of

A

anti-phospholipid antibodies

41
Q

cryoglobulin is typically what immunoglobulin dominant

A

IgM

42
Q

cryoglobulin pattern

A

short vague substructure or is microtubular

43
Q

immunotactoid glomerulopathy (GP) pattern

A
  • clonal staining

- microtubular substructure

44
Q

fibrillary glomerulonephritis (GN) pattern

A
  • polyclonal with proliferation

- randomly arranged fibrils

45
Q

hallmarks of granulomatosis w/ polyangiitis

A
  • older age
  • sinusitis
  • intermittent otitis media
  • crusting in nose
  • nodule on CXR
  • dysmorphic rbcs
  • AKI
  • peripheral edema
  • normal complements
46
Q

auto-Ab in granulomatosis w/ polyangiitis

A

proteinase 3–positive anti–neutrophil cytoplasmic autoantibody

47
Q

strongly associated with AR mutations in APOL1 gene in individuals of African ancestry

A
  • FSGS 

  • HIVAN
  • collapsing glomerulosclerosis a/w SLE 

  • SCD-associated nephropathy
48
Q

best test with highest sensitivity and specificity to evaluate for IG-related disorders

A

nephelometric assay for serum free light chains

49
Q

serum protein electrophoresis will miss cases due to its lower sensitivity

A

monoclonal diseases

50
Q

serum immunofixation electrophoresis will miss diseases that only produce

A

lights chains

51
Q

urine protein/immunofixation electrophoresis is not as sensitive as serum tests for

A

monoclonal proteins

52
Q

bevacizumab MOA

A

MAB against VEGF (vascular endothelial growth factor)

53
Q

HTN in setting of bevacizumab, renin and aldosterone levels

A

LOW

54
Q

best treatment for HTN and proteinuria 2/2 bevacizumab as long as no TMA

A

dihydropyridine calcium channel blocker (CCB)

55
Q

what does pamidronate nephrotoxicity cause?

A

collapsing FSGS and acute tubular injury

56
Q

clinical scenario diagnostic of classical polyarteritis nodosa (PAN)

A
  • severe HA
  • visual changes
  • fatigue
  • unintentional weight loss
  • fever
  • HTN
  • schistocytes on PBS
57
Q

renal arteriogram findings in classical polyarteritis nodosa (PAN)

A
  • multiple aneurysms
  • irregular constrictions in larger vessels
  • occlusion of smaller penetrating arteries
58
Q

clinical scenario consistent with TMA from gemcitabine

A
  • edema
  • livedo-like rash with erythematous ulcer
  • HTN
  • AKI
  • anemia
  • elevated LDH
  • proteinuria
  • hematuria
59
Q

cause of “anticoagulant nephropathy”

A

warfarin (glomerular bleeding)

60
Q
  • kappa light chains
  • nephrotic proteinuria
  • nodular glomerulus, and
  • granular deposition along BM and in nodule on EM are diagnostic of
A

LCDD

61
Q
  • lambda light chains
  • nephrotic proteinuria
  • nodular glomerulus, and
  • fibrillar deposition in the nodule on EM are diagnostic of
A

renal AL amyloidosis

62
Q
  • vasculitic skin rash
  • renal insufficiency
  • hematuria
  • nephrotic range proteinuria, and
  • low C4 complement are diagnostic of
A

cryoglobulinemia type 2 leading to MPGN with immune deposits

63
Q

diffuse podocyte effacement on EM and segmental scarring on light microscopy

A

FSGS

64
Q

diffuse podocyte effacement on EM and a normal light microscopy

A

MCD

65
Q

numerous subepithelial deposits on EM and spikes on silver stain

A

membranous nephropathy

66
Q

10 nm fibrils detected on EM and mesangial expansion with amorphous material on light microscopy

A

amyloid

67
Q

subendothelial deposits on EM and mesangial hypercellularity on light microscopy

A

membranoproliferative GN

68
Q
  • screening test for SLE w/ 95% sensitivity

- not diagnostic w/o other Abs or clinical features

A

ANA

69
Q
  • high specificity for SLE

- sensitivity 70%

A

anti-dsDNA Ab

70
Q
  • MOST specific Ab for SLE

- sensitivity only 30-40%

A

anti-Smith Ab

71
Q
  • present in 15% of patients w/ SLE

- seen w/ other connective tissue d/o’s, eg Sjogren syndrome

A

anti-Ro/SSA