SLE

Question 1

malar rash vs discoid rash

Answer 1

Malar rash – fixed erythema, flat or raised, over malar eminences, sparing nasolabial folds

Discoid rash – erythematous raised patches with keratotic scaling and follicular plugging; can cause atrophic scarring

Question 2

malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, and serositis (pleuritic pain or rub) or pericarditis can all be seen with ?

Answer 2

SLE

Question 3

is ANA specific to SLE?

Answer 3

ANA is positive for most SLE but +ANA is also seen in other diseases

Question 4

two antibodies most diagnostic for SLE?

Answer 4

anti dsDNA and anti Sm

Question 5

anti histone indicates?

Answer 5

drug induced SLE

Question 6

anti SSA (ro) and anti SSB (la)

Answer 6

seen with sjogrens syndrome

Question 7

anti SCL70 ab?

Answer 7

diffuse systemic sclerosis

Question 8

anti-centromere ab?

Answer 8

limited scleroderma (CREST syndrome)

Question 9

Antiphospholipid antibodies (to phospholipid B2 glycoprotein complex) also bind to…

and implies what false test?

Answer 9

cardiolipin and lupus anticoagulant

Interfere with in vitro PTT (lupus anticoagulant)

false + syphilis

Question 10

SLE peak onset

Answer 10

20-40 yo

F>M

Question 11

SLE genetics

Answer 11

Multi-gene inheritance and big environmental factor role

Specific HLA-DQ alleles linked to anti-dsDNA/anti-Sm;

Deficiencies in C2, C4, or C1q –> failure to clear immune complexes and apoptotic cells

• GWAS = lymphocyte signaling and IFN responses

Question 12

SLE environmental triggers

Answer 12

• UV light – induce apoptosis and stimulate IL-1 in keratinocytes

• sex hormones influence immune response.
Kleinfelter’s (XXY) increased incidence in males.

• Drugs – hydralazine, procainamide, D-penicillamine, INH. Reversible disease when drug induced.

Question 13

in SLE increased cytokines is due to…

treat w what drug?

Answer 13

increased BAFF = B cell survival

belumimab (anti BAFF)

Question 14

IFN elevated in SLE?

Answer 14

IFN1 alpha

Question 15

lupus susceptibility model

Answer 15

• Susceptibility genes impair self tolerance allowing external environmental triggers to trigger disease. Poor clearance results in persistence of nuclear antigens –> continued immune response
• Problem: env triggers are common yet SLE is not; predisposing genes are common normal alleles (not mutations or null loci)

Question 16

treatment options for lupus

Answer 16

• Corticosteroids (high activity) vs NSAIDs
• Anti-malarials for skin and organ involvement
• Cyclophosphamide for renal involvement

Question 17

common causes of death in lupus

Answer 17

renal failure, intercurrent infections, and coronary artery disease (atherosclerosis)

o Coronary artery disease= steroids accelerate the disease

Question 18

drug induced lupus

Answer 18

• Causative drugs: hydralazine, procainamide, isoniazid, and D-penicillamine

Symptoms: fever, arthritis, and serositis
• NO renal or CNS involvement
• ANA+ and + anti-histone Abs
• Normal serum complement
• Treatment: stop the drug! The disease will resolve!

Question 19

neuropsychiatric features of SLE

Answer 19

o headaches, seizures, neuropathy, stroke, chorea, and retinopathy (vasculitis), nerve palsy
o psychosis, depression, organic brain syndrome

Question 20

renal features of SLE

Answer 20

immune complex mediated
o Urinalysis has proteinuria and hematuria
o Serum creatinine increased with renal insufficiency
o High titers anti-dsDNA associated with active renal disease
o 6 classes of Lupus Nephritis

Question 21

6 classes of lupus nephritis

Answer 21

1 - Minimal mesangial – IC in mesangium, normal light microscopy
2 - Mesangial proliferative – increased mesangial cells and matrix with granular Ig, complement deposition. Normal gloms

3 - Focal LN - thickened capillary wall (wire loop fibrin deposits); renal insufficiency

5 – Membranous – subepithelial IC –> thickened capillary walls; severe proteinuria and nephrotic syndrome
6 - Advanced sclerosing - >90% gloms sclerosed

Question 22

musculoskeletal features of SLE

Answer 22

o Arthralgias and or arthritis – very common feature of SLE!
 Small joints, symmetric, non erosive synovitis
 Nondeforming
 Swelling and joint pain out of proportion to PE
o Myopathy - Muscle pain and weakness
o Arthritis

cardiac vegetations/endocarditis

Question 23

subacute cutaneous lupus erythematous

Answer 23

multiple skin lesions with reddened raised borders, central clearing, and light scale, non scarring, widespread
 Sun exposed areas like trunk and extremities but not on the face
 Associated with anti-SSA/Ro and HL-DR3 genotype

Question 24

chronic discoid lupus erythematous

Answer 24

face and scalp with thick scales, atrophy, scarring, edema and erythema
 Discoid lupus = coin and round shaped erythematous plaques with adherent scale
• Hyperpigmentation and depressed central scarring, atrophy, and depigmentation; usually face and scalp
• Lesion area shows hyperkeratosis – atrophic epidermis with areas of hyperplasia and large lymphoid infiltrates
• ANA+, dsDNA neg, + lupus band test

Question 25

skin features of SLE

Answer 25

o Ig/complement at dermal-epidermal junction (line of fluorescence at junction) = not specific but highly suggestive
 Lymphocytic infiltration, liquefaction of basal layer in epidermis, edema/perivascular lymphocytes in dermis
o Malar erythema (butterfly face rash) – similar on extremities and trunk; spares naso-labial folds; accentuated by sunlight
o Facial erythema – peeling can also occur

o Chronic discoid lupus erythematosus
o Subacute cutaneous lupus erythematosus

o	Ulcerations (oral, vaginal, nasal)
o	Alopecia (atypical hair loss)
o	Raynauds phenomena (arterial vasospasm in digits of hands and feet in response to cold or emotional stress)
o	Vasculitis (cutaneous and small vessel involvement)

Question 26

o Lupus Band Test

Answer 26

immunoglobulin and complement deposition in non-sun-exposed skin –> horizontal band of granular green immunofluorescence at the D-E junction shows deposits of IgM and c3b

Question 27

common constitutional features of SLE

Answer 27

fever, fatigue, and weight loss