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Ch 32 Secondary GN > SLE > Flashcards

SLE Flashcards

1
Q

What 2 populations where SLE is more often associated with severe nephritis?

A
  1. CHILDREN
  2. MALES
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2
Q

What are the 2 entry criteria for renal involvement in LN?

A
  1. PROTEINURIA
    >500mg/dL/day or 3+
  2. CELLULAR URINARY CASTS
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3
Q

What class is Minimal mesangial LN?

A

CLASS I

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4
Q

What class is Mesangial proliferative LN?

A

CLASS 2

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5
Q

What class is Focal LN?

A

CLASS 3

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6
Q

What class is Diffuse LN?

A

CLASS 4

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7
Q

What class is Membranous LN?

A

CLASS 5

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8
Q

What class is advanced sclerosing LN?

A

CLASS 6

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9
Q

What LN classes has active, chronic, active & chronic lesions?

A

CLASS 3 & 4

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10
Q

What Class of LN is characterized by:
LM - normal-appearing glomeruli
IF/EM - mesangial immune deposits

A

CLASS 1

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11
Q

What Class of LN has
IF/EM - purely mesangial hypercellularity, with mesangial immune deposits

A

CLASS 2

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12
Q

What class is defined as focal segmental and/or global endocapillary and/or extracapillary glomerulonephritis affecting < 50% of the total glomeruli sampled?

A

CLASS 3

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13
Q

What class is defined as focal segmental and/or global endocapillary and/or extracapillary glomerulonephritis affecting > 50% of the total glomeruli sampled?

A

CLASS 4

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14
Q

Class 4 lesions may have similar features with what type of GN?

A

MPGN

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15
Q

What Class of LN is defined by regular subepithelial immune deposits producing a membranous pattern with the coexistence of mesangial immune deposits and mesangial hypercellularity?

A

CLASS 5

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16
Q

What differentiates Primary MN vs Membranous LN

A

Primary MN - SUBEPITHELIAL IMMUNE COMPLEX DEPOSITS only

Membranous LN -
SUBEPITHELIAL IMMUNE COMPLEX DEPOSITS + MESANGIAL IMMUNE DEPOSITS & HYPERCELLULARITY

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17
Q

Wwhat Class of LN is characterized by > 90% of the glomeruli sclerotic and no residual activity?

A

CLASS 6

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18
Q

What class is characterized by mild global mesangial hypercellularity?

A

CLASS 2 LN

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19
Q

What Class is characterized by focal segmental endocapillary proloferation?

A

Class 3

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20
Q

What class is characterizwd by global endocapillary proliferation with infiltrating neutrophils & segmental wore loop deposits?

A

CLASS 4

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21
Q

What staining on IF is highly suggestive of LN?

A

“FULL HOUSE” STAINING

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22
Q

What is meant by “FULL HOUSE” staining on IF?

A

Presence of:
1. ALL 3 Igs: IgG, IgA, IgM
2. 2 COMPLEMENTS: C3, C1q

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23
Q

Describe the deposits seen on EM in LN?

A

• electron-dense
• granular
• “fingerprint” substructure
• tubuloreticular inclusions

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24
Q

What are the intracellular branching tubular structures measuring 24 nm in diameter located within dilated cisternae of the endoplasmic reticulum of glomerular and vascular endothelial cells, commonly observed in SLE biopsies?

A

TRIs

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25
TRIs are present in what 3 disease conditions?
1. SLE 2. HIV 3. VIRAL INFECTIONS
26
What are the 6 histologic features for Activity index?
1. Endocapillary hypercellularity 2. Glomerular neutrophil infiltration/Karyorrhexis 3. Interstitial inflammation 4. Wire loop lesions 5. Fibrinoid necrosis 6. Fibrocelullar/cellular crescents
27
What is the maximum activity incex score?
24
28
What is the maximum chronicity index score?
12
29
What are the 2 severe lesions and assigned double weight in activity index score?
1. Fibrinoid necrosis 2. Crescents
30
What are the 4 histologic features for chronicity index?
1. GLOMERULOSCLEROSIS (focal/segmental) 2. FIBROUS CRESCENTS 3. TUBULAR ATROPHY 4. INTERSTITIAL FIBROSIS
31
What acitivity and chronicity index score is associated with a poor 10-year renal survival rate?
High Activity index > 12 High Chronicity index >4
32
What 4 inflammatory infiltrates can be seen in active tubulointerstitial lesions?
1. T LYMPHOCYTES 2. B LYMPHOCYTES 3. MONOCYTES 4. PLASMA CELLS
33
What are the 2 lab findings in active lupus serology?
1. HIGH ANTI-DNA TITER 2. LOW SERUM COMPLEMENT
34
What Class of LN present with the most active clinical features?
CLASS 4
35
What Class of LN is predisposed to thrombotic complications such as renal vein thrombosis and pulmonary emboli?
CLASS 5
36
What Class of LN where levels of anti-DNA antibodies and serum complement levels often normalize?
CLASS 6
37
What are the 4 antibodies found in SLE?
1. ANA (against nuclear antigens) 2. ANTI-DNA (against DNA) 3. ANTI-dsDNA (against dsDNA) 4. ANTI-ssDNA (against ssDNA)
38
What are the 2 antibodies included in the ACR criteria?
1. ANA 2. ANTI-DNA
39
Wjat are the 2 antibodies used yo monitor course of SLE?
1. ANA 2. ANTI-DNA
40
What is a highly sensitive screening teat for SLE?
ANA
41
What is a more specific test for SLE?
ANTI-dsDNA
42
High titers of this antibody correlates well with clinical activity?
ANTI-dsDNA
43
What antibody correlates best with renal disease in SLE?
ANTI-dsDNA
44
What are the 4 antibodies directed against ribonuclear antigens?
1. ANTI-SM (against ENA) 2. ANTI-nRNP (against ENA) 3. ANTI-Ro/SSA (against cytoplasmic RNA) 4. ANTI-La/SSA (against nuclear RNP)
45
What is the most specific antibody for SLE?
ANTI-SM
46
What antibody is important in the pathogenesis of neonatal lupus and cardiac conduction abnormalities in the newborn?
MATERNAL ANTI-Ro/SSA
47
What antibody is associated with a unique dermal psoriasiform type of lupus?
ANTI-Ro/SSA
48
Levels of which decline before SLE flare?
C3, C4
49
What are the 3 lab test used to predict "flare" of SLE/LN?
1. C3 (low) 2. C4 (low) 3. ANTI-DNA Titer (high)
50
What drug for LN is contraindicated during pregnancy because of it's teratogenicity?
CYCLOPHOSPHAMIDE
51
What are the 4 drugs for SLE that can been used during pregancy?
1. CORTICOSTEROIDS 2. AZATHIOPRINE 3. CYCLOSPORINE 4. TACROLIMUS
52
What are the 3 risk factors that confers high risk for fetal loss durimg prenancy in LN?
1. ANTICARDIOLIPIN/APL ANTIBODIES 2. HPN 3. HEAVY PROTEINURIA
53
What is the duration of dialysis that patients with active SLE should undergo to allow clinical and serologic disease activity to become quiescent before transplantation?
3-12 months
54
What are the 6 medications used in the Induction therapy in LN?
1. CORTICOSTEROIDS (IV/Oral) 2. CYCLOPHOSPHAMIDE (IV/Oral) 3. MMF 4. CYCLOSPORINE 5. TACROLIMUS 6. RITUXIMAB
55
What is the most widely used induction immunosuppressive regimen for LN?
CORTICOSTEROIDS + CYCLOPHOSPHAMIDE
56
Cyclophosphamide SE is least severe in what form? (IV or Oral)
IV
57
What is the protocol used for IV cylophosphamide for induction therapy for LN?
Once a month x 6 months
58
What are the 3 least common SE with IV Cyclophosphamide?
1. HEMORRHAGIC CYSTITIS 2. ALOPECIA 3. NEOPLASMS
59
What are the 2 mcm SE of IV Cyclophosphamide?
1. MENSTRUAL IRREGULARITIES 2. PREMATURE MENOPAUSE
60
What cytoprotective agent is used to reduce bladder complications from Cyclophosphamide?
MESNA
61
What drug is comparable to cyclophosphamide for the treatment of LN?
MMF
62
What 2 regimens is supported by ACR & (KDIGO) guidelines as first-line therapy for severe LN?
1. CYCLOPHOSPHAMIDE 2. MMF
63
What 2 drugs are recommended by ACR & KDIGO for maintenance therapy for LN?
1. MMF 2. AZATHIOPRINE
64
What is a chimeric monoclonal antibody which depletes CD20 B cells?
RITUXIMAB
65
What is the drug used in patients resistant to other treatments and in those who do not tolerate conventional treatment?
RITUXIMAB
66
What therapy is used for LN patients with: 1. severe pulmonary hemorrhage 2. TTP-like syndrome 3. Those with APL antibodies and a clotting episode who cannot be anticoagulated due to hemorrhage
PLASMAPHERESIS
67
What therapy is used to treat thrombocytopenia in LN?
IVIG
68
Class 5 MN should be treated with this 4 therapies?
1. ANTI-HPN 2. ANTI-PROTEINURIC 3. LIPID LOWERING AGENTS 4. ANTICOAGULANT (should be individualized)
69
What 3 drugs are used to prevent glucocorticoid-induced osteoporosis?
1. CALCIUM 2. VIT. D SUPPLEMENTS 3. BISPHOSPHONATES
70
Plasmapheresis is used for what 3 groups of LN patients?
1. Pulmonary hemorrhage (severe) 2. TTP-like syndrome 3. Those with APL antibodies and a clotting episode who cannot be anticoagulated due to hemorrhage
71
What are the 2 antibodies against ENA?
1. ANTI-SM 2. ANTI-nRNP
72
What is the antibody against cytoplasmic RNA?
ANTI-Ro/SSA
73
What is the antibody against nuclear RNA?
ANTI-La/SSA

Ch 32 Secondary GN (35 decks)

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