lupus + other MCTD

Question 1

fact: antinuclear antibodies (ANA) are autoantibodies that target various nuclear antigens

Answer 1

FACT! *May be detected by ELISA (new) or IIF (old)

Question 2

Ro/SSA

Answer 2

Sjogren, neonatal lupus, SCLE, SLE

Question 3

La/SSB

Answer 3

Sjogren, SCLE

Question 4

Scl-70

Answer 4

diffuse systemic scleroderma

Question 5

Jo-1

Answer 5

dermato/poly-myositis

Question 6

anti-Smith

Answer 6

SLE

Question 7

U1RNP

Answer 7

MCTD

Question 8

dsDNA

Answer 8

SLE a/w lupus nephritis and correlates with lupus band test

Question 9

histone

Answer 9

drug induced SLE; linear and generalized morphea

Question 10

anti-centromere

Answer 10

CREST

Question 11

best screening test for systemic autoimmune connective tissue disorders?

Answer 11

ANA

Question 12

What are the associated diseases based on ANA IIF patterns? (1) homogenous (2) peripheral aka “rim” (3) speckled (4) nucleolar (5) centromere

Answer 12

(1) SLE, drug induced SLE
(2) SLE
(3) Sjs, MCTD
(4) Systemic Scleroderma
(5) CREST

Question 13

how many patients with DLE will progress to SLE?

Answer 13

5-20%

highest risk is in patients with diffuse DLE and in children with DLE

Question 14

epidemiology for DLE and SCLE

Answer 14

African American patients (DLE) and Caucasian (SCLE)

Question 15

risk factors for lupus

Answer 15

genetics, smoking, UV exposure

Question 16

subtypes of lupus

Answer 16

(1) ACLE (2) SCLE (3) CCLE -> DLE, hypertrophic LE, lupus tumidus, lupus panniculitis, lupus profundus, chillblain LE, neonatal LE

Question 17

hypertrophic LP vs. hypertrophic LE

Where are they found on the body clinically?

Answer 17

LP - affects lower 1/2 of body
LE - affects upper 1/2 of body

**E before P

Question 18

increased risk of ___ cancer in hypertrophic LE and DLE?

Answer 18

SCC

Question 19

location of chilblain LE?

Answer 19

fingertips, rims of ears, calves, and heels. FYI: Precipitated by cold, but may persist year round. demonstrates peri-eccrine inflammation on path

Question 20

anti-ssDNA

Answer 20

linear morphea; increased risk of SLE in DLE patients

Question 21

anti-C1q

Answer 21

hypocomplementemic urticarial vasculitis; SLE w/ nephritis

Question 22

anti-cardiolipin

Answer 22

antiphospholipid syndrome in SLE

Question 23

beta 2 glycoprotein

Answer 23

high risk of thrombosis in SLE; antiphospholipid syndrome in SLE

Question 24

anti-rRNP

Answer 24

neuropsychiatric LE

Question 25

anti-jo1 anti-PL7 anti-PL12 anti-EJ/OJ

Answer 25

anti-synthetase syndrome (***mechanic's hands) in DM

Question 26

anti-Mi2

Answer 26

good prognosis in DM

Question 27

anti-MDA5/CADM-140

Answer 27

DM with ILD

Question 28

p155/140 or T1F1

Answer 28

DM with cancer; amyopathic DM

Question 29

anti-SAE

Answer 29

adult amyopathic DM

Question 30

anti-RNA polymerases I and III

Answer 30

Systemic Sclerosis w/ severe skin involvement AND renal crisis**

Question 31

anti-fibrillarin (U3RNP)

Answer 31

Systemic Sclerosis a/w internal organ involvement

Question 32

alpha fodrin

Answer 32

Sjogren's (most specific)

Question 33

RF (rheumatoid factor)

Answer 33

rheumatoid arthritis; mixed cryoglobulinemia II and III

Question 34

cyclic citrullinated protein (CCP)

Answer 34

rheumatoid arthritis

Question 35

Do I affect the epidermis, dermis, or subq? (1) ACLE (2) SCLE (3) lupus tumidus (4) lupus panniculitis

Answer 35

1. upper dermis 2. upper dermis 3. lower dermis 4. subq

Question 36

Is lupus panniculitis septal or lobular?

Answer 36

lobular panniculitis

Question 37

what is the treatment algorithm?

Answer 37

start with topical treatments (CS or CI), then progress to antimalarials and lastly, oral prednisone, MTX, Retinoids, daponse, cellcept, cyclophosphamide

Question 38

SCLE is a/w complement deficiencies...which ones?

Answer 38

C1q/r/s, C2, and C4 FYI: C2 is the most common cause of complement deficiency associated lupus FYI: C2 is a/w less severe disease, C1 and C4 severe and/or recalcitrant disease FYI: C1q a/w lupus nephritis

Question 39

which drugs cause drug-induced SCLE? SLE?

Answer 39

- #1 HCTZ followed by terbinafine, griseofulvin, NSAIDs, calcium channel blockers, antihistamines, proton pump inhibitors, ACEi, TNF inhibitors (e.g. etanercept), and docetaxel - procainamide and hydralazine, methyldopa, d-penicillamine, minocycline, TNF inhibitors (e.g. etanercept, infliximab)

Question 40

neonatal lupus

Answer 40

lesions often present on the face, scalp, and neck. either at birth or in first few days of life. + telangiectasia/dyspigmentation. +anti Ro/SSA like SCLE. other findings which may be present at birth --> IRREVERSIBLE 3rd degree heart block, hematologic cytopenias, elevated bilirubin/ALT/AST. 2/3 of patients will need pacemaker.

Question 41

Tx for women whose babies are at increased risk of developed neonatal lupus

Answer 41

prenatal oral steroids (decrease cardiac risk in neonate) OR plaquenil throughout pregnancy (decrease cardiac and skin risk)

Question 42

HLA associations in SCLE

Answer 42

HLA-B8*** or HLA-DR3 FYI: these are also seen in palmoplantar psoriasis

Question 43

what is the lupus band test?

Answer 43

granular continuous band of immunoglobulin deposits and complement (C3) at DEJ in lesion and non-lesional skin in sun exposed/protected skin in patients with SLE

Question 44

Bullous SLE

Answer 44

predominantes in African-Americans, Ab to collagen 7 like in EBA, sub epidermal bullae with PMNs at DEJ, salt split skin: dermal side, Tx: dapsone

Question 45

Rowell's syndrome

Answer 45

ACLE/SCLE/DLE + erythema multiforme

Question 46

TEN-like lupus

Answer 46

triggered by high levels of UV exposure in patients w/ pre-existing ACLE or SCLE

Question 47

Which types of cutaneous lupus patients are at increased risk of developing SLE?

Answer 47

ACLE

Question 48

common causes of death in < 5 years? > 5 years?

Answer 48

inflammatory lesions of SLE and infx; thromboses

Question 49

drug-induced SLE

Answer 49

often no skin findings and mild systemic involvement; often procainamide or hydralazine; however, with minocycline, positive ANCA and negative histone Ab. And with TNF-alpha inhibitors, + dsDNA > + histone and increased skin involvement

Question 50

lupus related diseases (these are on a spectrum with lupus tumidus)

Answer 50

(1) jessner lymphocytic infiltrate of the skin | 2) reticular erythematous mucinosis (REM

Question 51

anti-SRP

Answer 51

DM or PM with cardiac involvement, poor prognosis