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rheum + derm > lupus + other MCTD > Flashcards

lupus + other MCTD Flashcards

1
Q

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

A

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

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2
Q

Ro/SSA

A

Sjogren, neonatal lupus, SCLE, SLE

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3
Q

La/SSB

A

Sjogren, SCLE

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4
Q

Scl-70

A

diffuse systemic scleroderma

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5
Q

Jo-1

A

dermato/poly-myositis

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6
Q

anti-Smith

A

SLE

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7
Q

U1RNP

A

MCTD

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8
Q

dsDNA

A

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

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9
Q

histone

A

drug induced SLE; linear and generalized morphea

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10
Q

anti-centromere

A

CREST

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11
Q

best screening test for systemic autoimmune connective tissue disorders?

A

ANA

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12
Q

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

A

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

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13
Q

how many patients with DLE will progress to SLE?

A

5-20%

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

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14
Q

epidemiology for DLE and SCLE

A

African American patients (DLE) and Caucasian (SCLE)

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15
Q

risk factors for lupus

A

genetics, smoking, UV exposure

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16
Q

subtypes of lupus

A

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

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17
Q

hypertrophic LP vs. hypertrophic LE

Where are they found on the body clinically?

A

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

**E before P

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18
Q

increased risk of ___ cancer in hypertrophic LE and DLE?

A

SCC

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19
Q

location of chilblain LE?

A

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

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20
Q

anti-ssDNA

A

linear morphea; increased risk of SLE in DLE patients

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21
Q

anti-C1q

A

hypocomplementemic urticarial vasculitis; SLE w/ nephritis

22
Q

anti-cardiolipin

A

antiphospholipid syndrome in SLE

23
Q

beta 2 glycoprotein

A

high risk of thrombosis in SLE; antiphospholipid syndrome in SLE

24
Q

anti-rRNP

A

neuropsychiatric LE

25
Q

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

A

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

26
Q

anti-Mi2

A

good prognosis in DM

27
Q

anti-MDA5/CADM-140

A

DM with ILD

28
Q

p155/140 or T1F1

A

DM with cancer; amyopathic DM

29
Q

anti-SAE

A

adult amyopathic DM

30
Q

anti-RNA polymerases I and III

A

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

31
Q

anti-fibrillarin (U3RNP)

A

Systemic Sclerosis a/w internal organ involvement

32
Q

alpha fodrin

A

Sjogren’s (most specific)

33
Q

RF (rheumatoid factor)

A

rheumatoid arthritis; mixed cryoglobulinemia II and III

34
Q

cyclic citrullinated protein (CCP)

A

rheumatoid arthritis

35
Q

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

A
  1. upper dermis
  2. upper dermis
  3. lower dermis
  4. subq
36
Q

Is lupus panniculitis septal or lobular?

A

lobular panniculitis

37
Q

what is the treatment algorithm?

A

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

38
Q

SCLE is a/w complement deficiencies…which ones?

A

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

39
Q

which drugs cause drug-induced SCLE? SLE?

A
  • # 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)
40
Q

neonatal lupus

A

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.

41
Q

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

A

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

42
Q

HLA associations in SCLE

A

HLA-B8*** or HLA-DR3

FYI: these are also seen in palmoplantar psoriasis

43
Q

what is the lupus band test?

A

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

44
Q

Bullous SLE

A

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

45
Q

Rowell’s syndrome

A

ACLE/SCLE/DLE + erythema multiforme

46
Q

TEN-like lupus

A

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

47
Q

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

A

ACLE

48
Q

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

A

inflammatory lesions of SLE and infx; thromboses

49
Q

drug-induced SLE

A

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

50
Q

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

A

(1) jessner lymphocytic infiltrate of the skin

2) reticular erythematous mucinosis (REM

51
Q

anti-SRP

A

DM or PM with cardiac involvement, poor prognosis

rheum + derm (13 decks)

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