SLE

Question 1

Abnormal immune responses in SLE include: (4)

Answer 1

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

Question 2

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

Answer 2

Upregulation of genes induced by Interferons

Question 3

DRVVT stands for

Answer 3

Dilute Russell viper venom time

Question 4

Best screening test I

Answer 4

ANA (Antinuclear antibody)

Question 5

SLE specific

May correlate with SLE activity (nephritis, vasculitis)

Answer 5

Anti-dsDNA

Question 6

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

Answer 6

Anti-Sm

Question 7

NOT specific for SLE

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

Answer 7

Anti-RNP

Question 8

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

Answer 8

Anti SS-A

Anti-Ro

Question 9

Associated with dec risk of nephritis

Answer 9

Anti-Ro(SS-A)

Anti-La (SS-B)

Question 10

Drug induced lupus marker

Answer 10

Antihistone

Question 11

Marker predisposes to thrombpcytopenia, fetal loss, clotting

Answer 11

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

Question 12

Measured as direct Coombs test; small proportion develops overt hemolysis

Answer 12

Anti erythrocyte

Question 13

May correlate to active CNS lupus

Answer 13

Antineuronal

Antiribosomal P

Question 14

Most common predisposing genes known

Answer 14

HLA DRB1 *0301 *1501

HLA DR3

Question 15

Female sex is permissive for SLE because

Answer 15

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

Question 16

Environmental stimuli may influence SLE

Answer 16

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

Question 17

Most common blood vessel pathology

Answer 17

Leukocytoclastic vasculitis

Question 18

Skin biopsy findings

Answer 18

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

Question 19

Classification of Lupus Nephritis:

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

Answer 19

CLASS I: Minimal Mesangial Lupus Nephritis

Question 20

Classification of Lupus Nephritis:

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

Answer 20

CLASS II: Mesangial PROLIFERATIVE Lupus Nephritis

Question 21

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

Answer 21

CLASS III: FOCAL Lupus Nephritis

Question 22

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

Answer 22

CLASS IV: DIFFUSE Lupus Nephritis

Question 23

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

Answer 23

CLASS V: MEMBRANOUS Lupus Nephritis

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

Question 24

Classification of Lupus Nephritis:

>/= 90% of glomeruli globally sclerosed

Answer 24

CLASS VI: Advanced SCLEROTIC Lupus Nephritis

Question 25

SLICC CLINICAL

Answer 25

``` 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

Question 26

SLICC IMMUNOLOGIC

Answer 26

``` ANA anti dsDNA Ant Sm Antiphospholipid Low serum complement Direct Coombs test (+) ```

Question 27

Most common chronic dermatitis in SLE

Answer 27

Discoid lupus erythematosus Only 5% have SLE And among SLE ONLY 20% have DLE

Question 28

Most serious manifestation of SLE

Answer 28

Nephritis

Question 29

Indicators of dangerous proliferative glomerular damage

Answer 29

Microscopic hematuria | Protenuria >500mg/24hr

Question 30

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

Answer 30

>/= 40mg prednisone and equivalent

Question 31

Most common manifestation of diffuse CNS lupus

Answer 31

Cognitive dysfunction (memory/reasoning)

Question 32

Most frequent hema manifestation

Answer 32

Anemia of chronic disease (normo- normo-)

Question 33

Diagnosis of APAS

Answer 33

1 or more clotting episodes and/OR repeated fetal loss 2 or more positive antiphospholipid tests at least 12 weeks apart

Question 34

SLEDAI score of active disease

Answer 34

Greater than 3

Question 35

Antimalarial that reduces flares and accrual of tissue damage over time

Answer 35

Hydroxychloroquine Side effect is retinal toxicity

Question 36

Amenorrhea, leukopenia and nausea as side effect: | MMF or cyclophosphamide?

Answer 36

Cyclophosphamide: an alkylating agent

Question 37

Diarrhea as side effect: | MMF or cyclophosphamide?

Answer 37

Mycophenolate mofetil: lymphocyte-specific inhibitor of IMP

Question 38

Pregnant with active SLE tx

Answer 38

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

Question 39

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

Answer 39

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

Question 40

SLE patient taking NSAID are more prone to this CNS complication

Answer 40

Aseptic Meningitis

Question 41

Quinacrine side effect

Answer 41

Diffuse yellow skin

Question 42

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

Answer 42

MTX: a folinic acid antagonist

Question 43

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

Answer 43

CYCLOPHOSPHAMIDE

Question 44

Lupus nephritis tx mainstay

Answer 44

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

Question 45

Rash Congenital heart block Cardiomyopathy

Answer 45

Neonatal lupus

Question 46

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

Answer 46

A. 2 to 2.5 | B. 3 to 3.5

Question 47

Lupus dermatitis SPF

Answer 47

30

Question 48

Lupus dermatitis tx

Answer 48

Topical sunscreen/ GC/ tacrolimus; and antimalarials Retinoic if not improved Systemic GC for extensive dermatitides-> overlap with a secon medication while tapering

Question 49

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

Answer 49

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

Question 50

True or False: All drug-induced lupus are ANA positive.

Answer 50

True

Question 51

SLICC 93% specific, 92% sensitive

Answer 51

>/= 4 criteria satisified, | atleast 1 clinical, 1 immunologic

Question 52

Topical sunscreens SPF

Answer 52

30