Lupus

Question 1

SLE is characterized by:

Answer 1

Polyclonal B-cell activation and abnormal autoantibodies

Question 2

Complement deficiencies associated with SLE:

Answer 2

C1q (rare but highest risk), C1r, C4, C2, C1 inhibitor deficiency, CR1 receptor deficiency

Question 3

Increased risk for SLE:

Answer 3

HLA-DR2 (anti-DNA Abs)
HLA-DR3 (anti-Ro Abs)
Null alleles at C2 and C4 loci
May be autosomal dominant

Question 4

SLE Criteria:

Answer 4

Malar Rash
Discoid Rash (can leave scars)
Photosensitivity
Oral Ulcers
Non-deforming arthritis, Serositis
Proteinuria >0.5 g/d
Neurologic disorders
Heme disorders
Anti-DNA, Sm, lupus anticoagulant, APS, false + RPR (antiphospholipid antibodies interfere w/ the test)
Positive FANA

Diagnosis requires 4 or more criteria

Question 5

Anti-histone antibodies are associated with:

Answer 5

SLE and drug-induced lupus

Question 6

What is test is required to be positive for the diagnosis of SLE?

Answer 6

ANA

Question 7

The chronic noninflammatory deforming arthropathy found in SLE patients.

Answer 7

Jaccoud’s arthropathy

Question 8

Immune complex-mediated damage in SLE:

Answer 8

glomerulonephritis

Question 9

Direct autoantibody-induced damage in SLE:

Answer 9

thrombocytopenia and hemolytic anemia

Question 10

Antiphospholipid antibody-induced damage in SLE:

Answer 10

Thrombosis and pregnancy morbidity

Question 11

BLYS-APRIL over-expression:

Answer 11

Increased IFNa, TNFa, IL-1, IL-6, IL-17

Question 12

Complement-mediated inflammation in SLE:

Answer 12

CNS lupus, hypoxemia, and anti-phospholipid mediated fetal loss

Question 13

Anti-ds DNA Abs can be useful for:

Answer 13

diagnosis, prognosis, therapeutic monitoring

Question 14

Anti-DNA, C3/C4 profile in SLE:

Answer 14

Increased Anti-DNA

Decreased C3/C4

Question 15

Non-infectious endocarditis in SLE:

Answer 15

Libmann-Sachs

Question 16

Leading causes of death in SLE:

Answer 16

1. Active lupus
2. Infection
3. Cardiovascular disease

Question 17

What lung disease is particularly common in pts with APS?

Answer 17

Alveolar hemorrhage (capillary thromboses break and cause hemorrhage)

Question 18

Most hematologic abnormalities in SLE are caused by:

Answer 18

Peripheral destruction of hematologic elements by complement, etc. Therefore, the BM is usually hyperplastic.

Question 19

Anti-Ro and Anti-La Abs cause:

Answer 19

neonatal lupus with congenital heart block
ANA negative lupus
Subacute cutaneous lupus erythematosus
C2 deficiency and lupus-like syndrome
DR3 gene association

Question 20

Lupus Treatment:

Answer 20

Vitamin D supplementation as an immunomodulator
Hydroxychloroquine
Corticosteroids (avoid in long-term)
Immunosuppressive agents
Targeted biologic therapies
Statisn (especially for APS)

Question 21

Hydroxychloroquine:

Answer 21

Prevents thrombotic events
Anti-platelet agent
Prevents lupus flare-ups
Lowers glycemia and lipids
Downregulates inflammation

Question 22

Why should NSAIDS be avoided in SLE?

Answer 22

Does not lower cardiac risks

Induces GI bleeding

Question 23

Drug-Induced Lupus:

Answer 23

Hydralazine, Procainamide, Quinidine, Isoniazid, etc.
Renal and CNS involvement are rare
C3/C4 levels are usually normal
DNA antibodies are rare
Anti-histone antibodies are commonly seen
Disappears within weeks of stopping the drug

Question 24

APS Lab diagnostic criteria:

Answer 24

Positive APS abs or anti-B2 glycoprotein 1 abs by ELISA on two or more occasions at least 12 weeks apart.