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MyDermatology-Bolognia > Ch. 41-Lupus > Flashcards

Ch. 41-Lupus Flashcards

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1
Q

What is the strongest risk factor for SLE?

A

Gender-Women6:1 ratio for sLE3:1 for cutaneous only

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2
Q

Name risk factors for sLE

A

female sexethnicity-africana American

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3
Q

Prevalence lupus in caucasian women? Africa-american women?

A

4 in 1000 AA1 in 1000 caucasian

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4
Q

How does lupus differ in African Americans compared to caucasians

A

-younger age-more severe disease-more internal organ involvement-higher mortality

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5
Q

What are some environmental factors contributing to lupus

A

UVRsmokingmedicationsviruses maybe-EBV*may be initial trigger in genetically susceptible host to cause damage and expose nuclear proteins to body

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6
Q

Name the genes most strongly associated with SLE

A

HLA-DR4PTPN22STAT4IRF5TREX1C1q

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7
Q

What genes are DLE associated with?

A

HLA-B8-DR3C2/C4 deficiency

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8
Q

What genes is associated with familial chilblain lupus?

A

TREX1=dysfunction of the exonuclease leads to an accumulation of IFN-stimulatory nucleic acids

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9
Q

What forms of lupus do autoantibodies play the strongest role?

A

SCLE

Neonatal lupus

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10
Q

Which antibodies are associated with SCLE?

A

Anti-ro (anti-SSA)

Anti-la (anti-SSB)

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11
Q

What are the 2 Anti-ro antibodies

A

anti-SSA/Ro60 and anti-SSA/Ro52

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12
Q

Which autoantibodies are associated with neonatal lupus

A

Anti-ro and anti-la

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13
Q

What role does SSA/Ro60 play in the body?

A

role in cell survival following UVR, possibly via binding to misfolded non-coding RNAs and targeting them for degradation

Deficiency/antibodies agains SSA thus may prevent this function

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14
Q

What role does SSA/Ro52 play?

A

SSA/Ro52 has a known regulatory role in inflammation, targeting both interferon regulatory factor 3 (IRF3) and IRF8 for degradationThus: deficiency in this is pro inflammatory

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15
Q

How does UVR cause lupus flare?

A

-UVR induces apoptosis, which leads to translocation of cellular and nuclear antigens -autoantiboides are thus exposed to the nuclear antigens and cause cascade inflammation

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16
Q

Which cell types make up the infiltrate seen in lupus?

A

CXCR3-positive CD4+ and cD8+ T cells (lymphocytic) immature plasmacytoid dendritic cells

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17
Q

Describe pathogenesis SLE

A
  1. nuclear antigens to be exposed due to lack of clearance of dead cells (e.g. complement factors help macrophages clear these dead cells via opsonization)
  2. Loss of self tolerance and sensitization to antigens–> causes immature APC to pick up antigen via TLR3. APC presents antigen to naive T helper cells4. IL-4 secreted promotes Th2 maturation
  3. Th2 promotes B-cell maturation and production
  4. Plasma cells produce auto-antibodies
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18
Q

How do autoantibodies produce damage in SLE

A

-Ag-Ab complex binds to Fc receptors of immune cells–> pro inflammatory cytokines like IFN and TNF-Ag-Ab complexes can bind directly to cells and cause complement activation and damage

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19
Q

Most important inflammatory cytokine in SLE?

A

IFN-alpha

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20
Q

What are the 3 specific cutaneous lesions of lupus? Others?

A

ACLE-acute cutaneous lupus

SCLE-Subacute cutaneous lupus

CCLE-Chronic cutaneous lupus: DLE, tumid lupus, lupus panniculitis, chilblains

Others:Rowells Syndrome, Bullous SLE

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21
Q

Which lupus forms have interface dermatitis

A

ACLE, SCLE, CCLE (only DLE, less likely other forms

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22
Q

Where does DLE typically occur on the body?

A

Head and neck–> face, scalp, ears.

Can be widespread, but unlikely to occur on the body without also lesions on head/neck

Can occur on muscosal surface

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23
Q

What level of the skin does DLE affect?

A

Superficial and deep dermis

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24
Q

What are the clinical features of DLE?

A

Erythema, scaly, round/annular plaques often with hypo pigmentation in centre with hyperpigmentation at border scarring, alopecia, atrophy, follicular plugging

Can be photo-distributed (head/neck) but no clear association between sun exposure and their development

firm/indurated

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25
What % DLE develop SLE
10-20% (mostly first 5 yrs)Mostly by mucocutaneous involvementCan have arthralgia in DLEHigher risk if disseminated lesions
26
Variant of DLE?
Hypertrophic DLE with significant hyperkeratosis, often on extensor arms
27
Clinical features SCLE?
Photosensitive Mostly photo exposed areas, mid face often spared, often involves lateral face, extensor arms, shoulders, chest, back 2 types: Annular: raised pink scaly borders with central clearing, or papular bordersPapulosquamous: chronic psoraisiform or eczematous Minimally palpable Can get hypo pigmentation or depigmentation but no scarring
28
What % SCLE are linked to medications?
20-30%
29
Medications associated with SCLE
“PTHIN DRAGS” PPI/PUVA Terbinafine/TNF-alpha inhibitors/Taxanes HCTZ (#1) IFN NSAIDS Diltirazem + other CCBS/BBs Ranitidine Acei, anti-epileptics Griseofulvin Statins
30
What % patients SCLE fulfills SLE criteria
30-50%
31
What antibody is most associated iwht SCLE? What % have it?
SSA/Anti-Ro Ranges 60-100%, on avg 70%
32
Clinical presentation of ACLE?
Malar distribution with sparring of nasolabial fold, can be widespread on body, spares knuckles Transient (hours to a couple weeks) and photosensitive, resolve with no scarring Erythema to intense edema, can develop some scale, may have papular component May show poikiloderma ( atrophy, telengiectasias, dyspigmentation (hypo/hyper), may have erosions
33
Lupus variants
ACLE, SCLE, DLE (3 major) Tumid lupus (CCLE) Lupus panniculitis (CCLE) Chillblains (CCLE) Bullous lupus Rowels Neonatal (SCLE)
34
Clinical appearance tumid lupus
Firm, edematous erythematous plaques, urticarial appearing, with no surface change Mostly on the face, can occur on trunk \*Some think the same as Jessners Photosensitive
35
Is tumid lupus associated with SLE?
Majority have no other organ involvement, no serologic markers 10% of those with SLE with have tumid lupus lesions
36
Clinical presentation Lupus panniculitis
Can start as erythematous indurated plaques, become atrophic and depressed over time situ lipoatrophy May develop DLE over top= lupus profundus Located: upper trunk, breasts, buttocks, thighs, upper arms, face scalp
37
Clinical presentation chilblains lupus
red-purple dusky papules and plaques on toes, fingers, sometimes nose or elbows, knees May just be ordinary chilblains concurrent with lupus \*over time may become discoid
38
What genes mutations lead to familial chilblains? What do they encode for ?
TREX1 and SAMHD1 \*Exonuclease and nuclease-→lead to accumulation nucleic acids
39
What is familial chilblains
Chilblains Arthralgia + ANA But usually no systemic dz
40
What is Aicardia-Goutieres
AR autoinflammatory disorder -recurrent sterile fevers, chilblains, developmental delay
41
What ab is most associated with neonatal lupus
Anti-Ro/SSA
42
How does neonatal lupus occur?
Mom with SCLE, anti SSA/RO +, transplacentally cross over
43
What % neonatal lupus are ro+?
Nearly 100% Also some u1RNP
44
How does neonatal lupus present
Essentially identical to sCLE (annular erythematous plaques) in adults, predominantly on head/neck though Photosensitive but can occur without it, can be present at birth No scarring but can have residual pigmentation and telengiectassis Can have internal manifestation
45
What are the internal manifestations of neonatal lupus
Heart block, with or w/o cardiomyopathy Hepatobiliary Cytopenias-esp. thrombocytopenia Other reports DIC, hydrocephalus
46
When does heart block present in neonatal lupus
At birth CM may develop a few months after birth but typically also at birth 20% mortality with CM
47
How does hepatobiliary present in neonatal lupus
Liver failure Conjugated hyperbilirubenmia Minor liver enzyme elevations
48
Workup for suspected neonatal lupus
EKG +- echo CBC Liver enzymes ad bili Repeat q2-3 months x 2-3x (until 9 months wish)
49
What are the different presentations bullous lesions in lupus?
1. Bullous lesions occurring in setting intense basal damage with ACLE or SCLE, rarely DLE. 2. Rarely, a dramatic, acute eruption similar to erythema multiforme major or toxic epidermal necrolysis (TEN) may occur in patients with preexisting ACLE or SCLE, or it may appear *de novo* 3. True bullous lupus (next slide)
50
What is bullous eruption of SLE?
Acquired blistering disorder in patients with SLE Vesicles and bull that hits path resembles DH with microabsesses in dermal papillae and neutrophils
51
Bullous SLE targets?
Immunoreactants are often found at the basement membrane zone and antibodies to type VII collagen have been detected in several patient
52
Most common organ systems in SLE
Joints Skin Renal CNS Heme Pleurisy Constitutional sX: Fevers, joint pain, weight loss, LAD, fatigue
53
Criteria for SLE
Need 4/11 1. Malar rash 2. Discoid rash 3. Photosensitivity 4. Oral ulcers 5. Arthritis (non erosive, 2 or more) 6. Serositis (pleurites or pericarditis) 7. Renal disorder-persistent proteinuria or cellular casts 8. Neurologic disorder-psychosis or seizures 9. Heme-→ hemolytic anemia, leukopenia \<4, lymphopenia \<1500, thrombocytop \<100 000, 10. Antibodies: anti-DNA, anti-sm, APLAS 11. + ANA
54
What are the non specific cutaneous findings in lupus?
1. Diffuse non scarring alopecia (or discoid lesions) 2. Raynauds 3. Periungual telengiectasias and erythema (less severe than Scleroderm or DM) 4. Vasculitis 1. urticarial vasculitis 2. small vessel vasculitis w/ palp purport 3. PAN-like lesions 4. ulcerations 5. Cutaneous signs APLAS 1. livedo 2. necrosis/ulcers 3. acrocyanosis 4. atrophie blanche 5. degos-like lesions 6. livedoid vasculopathy 6. Palmar erythema and erythromelaglia 7. Papular and nodular mucinosis 8. Sweets-syndreom of SLE 9. Anetoderma
55
What % patients with papulonodular mucinosis have SLE?
75%
56
livedo reticularis with ischemic CNS disease has been called?
Sneddon syndrome
57
What are the core Histopathology findings in lupus?
1. basal cell damage (interface dermatitis, vacuolar change) 2. Lymphohistiocytic infiltrate 3. Dermal Mucin deposition 4.
58
Name 5 pathological findings in acute cutaneous lupus
1. **basal cell vacuolization** 2. Superficial lymphocytic infiltrate 3. Apoptotic keratinocytes 4. Dermal mucin 5. Dermal edema 6. focal hemmorhage In general dermal changes can be quite subtle
59
Name 7 pathologic findings of SCLE
1. Basal cell vacuolization 2. apoptotic keratinocytes 3. superficial lymphocytic infiltrate (upper dermis) 4. Epidermal atrophy 5. Dermal mucin 6. Dermal edema 7. In general compared to DLE less (but still some): follicular plugging, hyperkeratosis, BMZ thickening Epidermal changes and superficial infiltrate are common
60
Name 9 findings DLE on path
1. Less vacuolar change 2. BMZ thickening 3. Follicular plugging 4. Superficial and deep lymphocytic infiltrate 5. Peri-adenxal infiltrate 6. Dermal mucin 7. Hyperkeratosis 8. Pilosebaceous atrophy 9. Dermal fibrosis
61
What does pathology of tumid lupus show
Prominent dermal mucin deposition and superficial and deep lymphocytic infiltrates with a lack of epidermal change
62
What does pathology for Lupus panniculitis show?
Lymphocytic infiltrate in subcutis, sometimes deep dermis and peri-adnexal maybe dermal mucin maybe epidermal atrophy sometimes vacuolar cahnge
63
What doe DIF for lupus show?
IgG or IgM granular deposits at dermal-epidermal junction, complement deposits May be epidermal in SCLE Though to be anti-ro/SSA \*Lesional skin
64
What does DIF in normal appearing skin show in SLE?
Ab deposits at DEJ, correlates with disease activity
65
Workup for suspected SLE patient
CBC BUn, Cr, UA ANA, anti-SSA/ro, SSB, ssDNA, histone, U1RNP, APLAS ESR/CRP Complement
66
How does drug induced lupus differ from SLE?
presents as a lupus-like systemic illness with arthralgias, myalgias, pleuritis and fever lacking nephritis and CNS disease Skin-typically no findings, sometimes malaria rash and photodistributed erythema
67
What ab is associated with drug induced SCLE?
anti-SSA/ro
68
what ab is associated with drug induced SLE?
anti-histone\*\* 95%
69
What drugs cause drug-induced SLE
"HIP MC" Hydralazine Isoniazid, interferon Procainamide, phenytoin, penicillamine (unmasks true dz) Minocycline (anti MPO), methyldopa, Chlorpromazine
70
Top 2 drugs known to cause SLE
HYdralazine Procainamide
71
What ab + in TNF-inhibitor induced SLE ? Wha tis characteristic tnf induced?
anti-dsDNA More CNS, more renal disease, more skin
72
Therapy for cutaneous lupus
1. sun protection, topical and intra-lesional steroids, topical tacrolimus, topical retinoids 2. Systemics: \*HCQ (200 mg po daily to BID), chloroquine, quinacrine (can be added) 3. Non anti-malarial systemics: oral retinoids, thalidomide, sulfasalazine, dapsone (bullous), MMF, AZA, MTX, ustekinumab, apremilast, systemic steroids
73
Max dose chloroquine
3.5–4 mg/kg *ideal body weight* /day \<2.3 mg/kg real weight/day for chloroquin
74
Max dose of hydroxychloroquine
5 mg/kg REAL weight/day 6.5 mg/kg ideal body weight
75
Main concerning side effect HCQ
Eye toxicity
76
Main side effect thalidomide
peripheral neuropathy
77
Treatment lupus nephritis
Pulse pred x 3 days MMF \>cycle
78
Treatment SLE
No organ-nsaids Mild organ-steroids Moderate: Pulse cychloposhamide +- pulsed steroids OR: HCQ, mycophenolate mofetil, azathioprine, methotrexate, leflunomide

MyDermatology-Bolognia (31 decks)

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