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Rheumatology Harrison's > SLE > Flashcards

SLE Flashcards

1
Q

Abnormal immune responses in SLE include: (4)

A
  1. Activation of innate immunity
  2. Lowered activation thresholds and abnormal activation pathways
  3. Ineffective regulatory CD4, CD8, T cells
  4. Reduced clearance if immune complexes and of apoptotic cells
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2
Q

Genetic signature in peripheral blood cells of 50-80% of SLE patients

A

Upregulation of genes induced by Interferons

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3
Q

DRVVT stands for

A

Dilute Russell viper venom time

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4
Q

Best screening test I

A

ANA (Antinuclear antibody)

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5
Q

SLE specific

May correlate with SLE activity (nephritis, vasculitis)

A

Anti-dsDNA

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6
Q

Specific for SLE
No correlation to activity
More common in blacks and asians more than whites
Most patients also have anti-RNP

A

Anti-Sm

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7
Q

NOT specific for SLE

high titers associated with syndromes that have overlap features of several rheumatic syndromes

A

Anti-RNP

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8
Q

Not specific for SLE
Sicca syndrome
Predisposes to subacute cutaneous lupus and to neonatal lupus with congenital heart block
Decreased risk for nephritis

A

Anti SS-A

Anti-Ro

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9
Q

Associated with dec risk of nephritis

A

Anti-Ro(SS-A)

Anti-La (SS-B)

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10
Q

Drug induced lupus marker

A

Antihistone

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11
Q

Marker predisposes to thrombpcytopenia, fetal loss, clotting

A

Antiphospholipid (cardiolipin, beta 2 glycoprotein 1, lupus anticoagulant)

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12
Q

Measured as direct Coombs test; small proportion develops overt hemolysis

A

Anti erythrocyte

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13
Q

May correlate to active CNS lupus

A

Antineuronal

Antiribosomal P

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14
Q

Most common predisposing genes known

A

HLA DRB1 *0301 *1501

HLA DR3

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15
Q

Female sex is permissive for SLE because

A

Higher antibody production
Exposure to estrogen pills or HRTs 1.2-2x risk
Hormones, genes on chromosome X

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16
Q

Environmental stimuli may influence SLE

A

UV light causes flare in 70% of patients
EBV
Current tobacco Smoking OR 1.5
Prolonged silica exposure

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17
Q

Most common blood vessel pathology

A

Leukocytoclastic vasculitis

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18
Q

Skin biopsy findings

A

Ig deposits at dermal epidermal junction
Injury to basal keratocytes
Inflammation dominated by T lymphocytes

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19
Q

Classification of Lupus Nephritis:

MESANGIAL immune deposits by immunofluorescence but normal glomeruli by light microscopy (LM)

A

CLASS I: Minimal Mesangial Lupus Nephritis

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20
Q

Classification of Lupus Nephritis:

MESANGIAL hypercellularity OR matrix expansion by LM, +mesangial immune deposits

A

CLASS II: Mesangial PROLIFERATIVE Lupus Nephritis

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21
Q

Classification of Lupus Nephritis:
Active or inactive focal, segmental, or global GN involving involving = 50% of glomeruli
WITH focal SUBENDOTHELIAL immune deposits
With or without mesangial alterations

A

CLASS III: FOCAL Lupus Nephritis

22
Q

Classification of Lupus Nephritis:
Active or inactive focal, segmental, or global GN involving involving >/= 50% of glomeruli
WITH diffuse SUBENDOTHELIAL immune deposits
With or without mesangial alterations

A

CLASS IV: DIFFUSE Lupus Nephritis

23
Q

Classification of Lupus Nephritis:
Global or segmental SUBEPITHELIAL immune deposits or sequelae by LM, IF or EM
With or without mesangial alterations

A

CLASS V: MEMBRANOUS Lupus Nephritis

May occur in combination with III or IV (still classified as V)
May show advanced sclerosis

24
Q

Classification of Lupus Nephritis:

>/= 90% of glomeruli globally sclerosed

A

CLASS VI: Advanced SCLEROTIC Lupus Nephritis

25
Q

SLICC CLINICAL

A 
Acute or chronic cutaneous Lupus
Oral or nasal ulcers 
Non scarring alopecia 
Arthritis
Serositis 
Neurologic seizures psychosis, mononeuritis
Hemolytic anemia
Leukopenia <4000 or lymphopenia <1000
Thrombocytopenia <100,000
Renal- protein:crea ratio >/= 0.5, RBC casts, biopsy

SOAP BRAIN

26
Q

SLICC IMMUNOLOGIC

A 
ANA
anti dsDNA 
Ant Sm
Antiphospholipid
Low serum complement 
Direct Coombs test (+)
27
Q

Most common chronic dermatitis in SLE

A

Discoid lupus erythematosus
Only 5% have SLE

And among SLE ONLY 20% have DLE

28
Q

Most serious manifestation of SLE

A

Nephritis

29
Q

Indicators of dangerous proliferative glomerular damage

A

Microscopic hematuria

Protenuria >500mg/24hr

30
Q

GC induced psychosis occurs during first weeks of treatment at what doses of steriods

A

> /= 40mg prednisone and equivalent

31
Q

Most common manifestation of diffuse CNS lupus

A

Cognitive dysfunction (memory/reasoning)

32
Q

Most frequent hema manifestation

A

Anemia of chronic disease (normo- normo-)

33
Q

Diagnosis of APAS

A

1 or more clotting episodes and/OR repeated fetal loss

2 or more positive antiphospholipid tests at least 12 weeks apart

34
Q

SLEDAI score of active disease

A

Greater than 3

35
Q

Antimalarial that reduces flares and accrual of tissue damage over time

A

Hydroxychloroquine

Side effect is retinal toxicity

36
Q

Amenorrhea, leukopenia and nausea as side effect:

MMF or cyclophosphamide?

A

Cyclophosphamide: an alkylating agent

37
Q

Diarrhea as side effect:

MMF or cyclophosphamide?

A

Mycophenolate mofetil: lymphocyte-specific inhibitor of IMP

38
Q

Pregnant with active SLE tx

A

Hydroxychloroquine [category D]
Prednisone/ prednisolone at lowest dose possible (deactivated by placental enzyme)
Azathoprine may be added [category D]

*MTX [category X]

39
Q

If to start azathioprine, screen for this enzyme deficiency or else: higher risk for bone marrow suppression

A

Thiopurine S-methyltransferase (TPMT): required to metabole 6-mercaptopurine product of azathioprine

40
Q

SLE patient taking NSAID are more prone to this CNS complication

A

Aseptic Meningitis

41
Q

Quinacrine side effect

A

Diffuse yellow skin

42
Q

May have a role in dermatitis, arthritis but probably not in

A

MTX: a folinic acid antagonist

43
Q

Has adverse effects of: hemorrhagic cystitis (less with IV), carcinoma of the bladder, VZV infection

A

CYCLOPHOSPHAMIDE

44
Q

Lupus nephritis tx mainstay

A

SYSTEMIC GC:
Methylprednisolone Na succinate 0.5-1 mg/kg/day OR 500-1000 mg IV x 3 days followed by
Prednisone 0.5-1 mg/kg/day PO x 4-6 weeks then tapered

45
Q

Rash
Congenital heart block
Cardiomyopathy

A

Neonatal lupus

46
Q

Target INR in lupus and APS
A. 1 episode of venous clotting
B. With RECURRENT clots

A

A. 2 to 2.5

B. 3 to 3.5

47
Q

Lupus dermatitis SPF

A

30

48
Q

Lupus dermatitis tx

A

Topical sunscreen/ GC/ tacrolimus; and antimalarials
Retinoic if not improved

Systemic GC for extensive dermatitides-> overlap with a secon medication while tapering

49
Q

Criteria likely to respond to BELIMUMAB (considered after other approaches fail or are not tolerated

A

SLEDAI at least 10
(+) anti-dsDNA
low serum complement
-> robust clinical activity

50
Q

True or False:

All drug-induced lupus are ANA positive.

A

True

51
Q

SLICC 93% specific, 92% sensitive

A

> /= 4 criteria satisified,

atleast 1 clinical, 1 immunologic

52
Q

Topical sunscreens SPF

A

30

Rheumatology Harrison's (12 decks)

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