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Chapter 51 connective tissue diseases Melissa > Lupus > Flashcards

Lupus Flashcards

1
Q

DLE whopeak onset Associations

A

F>M4th decade in F, later in malesC2 deficiency in females associated. 14-26% raynauds, 30% anaemia, leukopenia, thrombocytopenia26% false +ve syphilis, 17% RF +ve1/3 alopecia 20% elevated ESR, increase in serum globulins

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2
Q

Disseminated DLE Who gets itWhat does it look like

A

Women, who usually smokePersistent, resistant to therapyMay look like papulosquamous SCLE but scarring occursreticulate telangiectasiaLE gyratum repents: associated with underlying malignancy, negative lupus band. Swindling of fingers+ hyperextension of distal phalanges

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3
Q

Factors associated with onset of DLE

A

Trauma, stress, UV exposure, Drugs , seasonal (summer> winter, premenstrual

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4
Q

Autoantibodies in DLE

A

5-60% +ve ANA (homogenous> speckled)More common in long disease, extensive, with raynauds, joint pains, chilblains0.-21% antidsDNA, 20% ssDNA (may represent widespread and progressive disease), 10% Ro Abs (no increased risk of SCLE), high incidence of antithyroid, parental cell Abs

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5
Q

DLENailsEyesMucous membranes

A

Nails: subungual hyperkeratosis, redblue nail plate, longitudinal striaeMucous membranes 24% involvement, LP like lesions, buccal mucosa, thickened rough lips, oral lesions may resemble leukoplakia Eye: velvety oedema, redness of conjunctiva. More common lower eyelids esp lateral 1/3. May be associated with scarring

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6
Q

Chillblain lupusWhoProgression to SLEAbs

A

Smokers, can occur years post DLE, and persist with tx when DLE remits. Usually precipitated by pregnancySome may have cryofibrinogenemia or cold agglutinins Neg lupus band Usually Ro +ve 15% get SLE, more common if develops DLE at same time and with EM.

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7
Q

Treatment of DLE

A

General measures- psyche, camouflage, photoprotectionTopical- potent topical steroids CS, often under occlusion-IL CS, CO2 laser, PDL for telangiectasia Oral- nifedipine for raynaudsPred for severe 0.5mg/kg, taper over 6/52HCQ 200mg bd then reduce, most respond by 6/.52stop smokingCan use thalidomide 100mg/day, cyclophosphamide

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8
Q

DDx of DLE

A

Morphea, LS, LPJessners/REMLupus vulgarisRosaceaNLDChilblainPMLEBlooms

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9
Q

Prognosis of DLE

A

SCC in 3.3%, scalp, ears, lip, nose >20 yearsScarring 57%scarring alopecia 35%Premenstrual flare 20%50% complete remission

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10
Q

Associations of DLE that make it more likely to be difficult to treat

A

raynauds, scalp, chilblain like lesions

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11
Q

Lupus panniculitis% with DLE clinically and histologically How does it heal

A

3-5% DLE clinically, 30% histologically Heals with anetoderma

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12
Q

Histology of lupus panniculitis

A

Epidermal atrophy, follicular plugging, hydropic degeneration of the basal layer. Lymphocytes around appendages and vessels in mid derma. Hyalinisation around fat cells. Lower dermal + SC necrobiosis with some vasculitis. Popular panniculitis. Fibrous spate and lymphoid folliculitis

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13
Q

Lupus panniculitis - associations

A

Trauma, biopsy, EMG, monoclonal gammopathy, thrombocytopenic purport

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14
Q

Usual sites of lupus panniculitis

A

Face, proximal extremities

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15
Q

Concomitant SLE in lupus panniculitis

A

10-20%

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16
Q

What do you have to histologically exclude from lupus pannicullitis

A

Subcutaneous panniculitis like T cell lymphoma

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17
Q

T/F immune deposits occur in DEJ in lupus panniculitis

A

T

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18
Q

ABs in Rowell’s

A

Speckled ANA, RF, anti La (SSB)

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19
Q

LE hypertrophicus et profundus

A

violaceous, scaly, tender lesions with a warty hypertrophic surface with scale and a brown-black tar like plaque. Extensive serological abnormalities Can resemble thrombophlebitis + calcification

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20
Q

% of DLE that progresses to SLE

A

Rook 5% (22% disseminated, 1.2% head and neck)BJD 16% progression within 3 years of DxBologna 5-10% Females

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21
Q

Does SCLE scar

A

Not usually, pigment changes

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22
Q

Drugs and SCLE

A

Griseofulvin, Antihistamines, Terbinafine, calcium channel blockers, HCT, NSAIDS, PPIs, antitnf.

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23
Q

% of SCLE with anti-ro

A

70% more likely if on face

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24
Q

ARA criteria (old for SLE)

A

ARA criteria - SOAP BRAIN MD (4 of 11)• Serositis (pleuritis/pericarditis)• Oral ulcers (painless)• Arthritis (2 or more peripheral joints – tenderness, swelling or effusion)• Photosensitivity• Blood (haemolytic anaemia with reticulocytosis, leukopenia 0.5g/day or more than 3+ /casts)• Antinuclear antibodies • Immunological disorder (LE cells or anti DNA or anti-Sm ab or antiphospholiopid abs (anticardiolipin, lupus anticoagulant, false +ve syphilis – longer than 6 months) • Neurological disorder (seizures/psychosis)• Malar rash (fixed erythema, spares nasolabial)• Discoid rash (follicular plugs, atrophic

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25
Most specific autoantibodies for SLE
antidsDNA and antiSmAbs
26
Ix for suspected SLE
- Examination: specific cutaneous lesions, non specific cutaneous lesions, lymphadenopathy, arthritis - Histology +/- lupus band- ANA with profile (anti-dsDNA, -Sm)- Urinalysis- eLFTS, FBC- ESR- C3, C4 - antiphospholipids - G6PD pre treatment
27
Tx SLE
Photoprotection o UVB is biggest problem (midday sun is high in UVB)Topicalso Topicals or IL steroids, tacrolimus +/- keratolytics Systemicso HCQ 6.5mg/kg/day +/- quinacrine (can turn skin yellow). Takes 2-3/12 for efficacy to be appreciated and several more months for maximal effect.  Smokers do not respond as well o CS, AZA, leflunomide, MMF o Pulse cyclophosphamide +/- pulse CS o Dapsone for bullous LE
28
Treatment of lupus panniculitis
clobetasol under occlusion, HCQ, ILCS, oral thalidomide
29
Tx of SCLE
photoprotectionTopical or IL CS, tacrolimusHCQ, oral CS or methylpredacitretin, isotretinoin, capstone, MTX, thalidomide, IFNalpha, MMF
30
NEW SLE criteria 4/17
At least 1 clinical + 1 immunologicalClinical: ACLE or SCLEChronic cutaneous lupusNon scarring alopeciaOral or nasal ulcersJoint diseaseserositisRenal NeurologicalHaemolytic anaemiaLeukopenia or lymphopeniaThrombocytopenia Immunological:ANA, antidsdna, anti-SM, antiphopholipid, low C3,C4, low CH50, direct cooks in absence of haemolytic anaemia
31
Antiphospholipids
Lupus anticoagulantanticardiolipinB2 glycoprotein
32
Antiphospholipid syndrome associated with
Libman sacks endocarditis, MI, valvular heart disease, a vascular necrosisThrombophlebitis, purport, lively, leg ulcers, gangrene, subungual splinter haemorrhages
33
HLA for annular phenotype of SCLE
HLA-DR3 (B8, other)
34
SCLE autoantibodies
60% homogenous ANA, 80% antiRo SSA, 16% anticardiolipin Ab
35
Histo of SCLE cf to DLE
More epidermal atrophy,k less hyperkeratosis, less BM thickening, less follicular plugging, and inflammatory infiltrationMore basal vascular change, dermal oedema and mucin
36
DIF of SCLE
More positive in papulosqumous 88% > angular 29%Overall 60%Granular in cytoplasm, nuclear of basal keratinocytes and in epidermis (unlike other variants)
37
Neonatal lupus % with skin lesionsPrecipitated by..Scarring?Residual lesions?
50%UV, phototherapy for jaundiceImproves in 1st few months, resolves without scarring by 12/12. May get residual telangiectasia, dyspigmentation ,atrophy, atrophic scarring.
38
Neonatal lupusRIsk of :Heart blockPacingDilated cardiomyopathyThrombocytopeniaMortality
Heart block 60%Pacing 50%Dilated cardiomyopathy 20%Thrombocytopenia 20% (transient)Mortality 20%
39
Other non haem or cardiac association with neonatal lupus
Liver: neonatal hepatitis, hepatomegaly + splenomegaly.
40
Ix of neonatal lupus
FBC, elfts - thrombocytopenia and liver involvementECG, echoCardiology reviewIf steroids given first 16/40, no conduction defectsHarm review - may need prod 2mg/kg/day
41
Risk of NLE in +ve Ro/SSA
2% if mother has no connective its use disease or previous hx of NLE 25% if previously NLE child
42
Precipitate of SLE
UVSprouts, seeds, alfalfa, heavy metals (cadmium, mercury, gold)Infection Premenstrual flare in 20%Drugs eg hydralazine, procainamide, TNFalpha, penicillamine, isoniazid, minocytcline.
43
Poor prognostic factors of SLE
Renal involvement more organs, males, Asians+ black, arthritis, antiphospholipid, thrombocytopenia, haemolytic anaemia and CNS involvement.
44
Good prognostic factors for SLE
CS, early Dx, decrease stress and drugs such as sulphonamides, control of infection with Abs
45
SLE usual initial presentation % with rash
58% articular 14% cutaneous 80% will have a rash
46
Clinical skin features of SLE
Specific (histo changes): erythema in photodistributed pattern, facial oedema, epidermal necrosis Nonspecific: reticulate telangiectasia erythema on thenar & hypothenar eminence of palms, pulps and dorsum fingersHyperkeratosis, ragged cuticlesHair: 50% alopecia: diffuse or scarring, 30% coarse, dry, fragile hair.
47
Significance of livedo outer arms in SLE
More frequently in CNS
48
Risk of membranous lupus nephropathy in SLE
8%
49
What is Senear-Usher
Pemphigus erythematosis (combines pemphigus + LE) ANA +ve 80-100%Pemphigus like Abs 80-100%DIF: Ig and complement in intercellular substance & at dermal, epidermal junction of perilesional skin Induced by penicillamine, captopril, propranolol
50
Bullous lupus histo
subepidermal blister with Neutrophils and microabscesses. Linear deposition of IgA, IgG and IgM, C3Type 1 (type VII collagen), 2 (other antigens) 3 (epidermal antigens)
51
Where does bullous LE occur
Face, neck, upper trunk1/3 have mouth lesions
52
Associations with bullous lupus
glomerulonephritisDrugs eg hydralazine, IFN alpha
53
T/F SLE is worse in childhood
T
54
SLE in elderly
Increase in incidence of lung disease and sjogrens 20% have onset >60 Decrease in renal and mesenteric vascultiis Increase in HLA DR3, frequent Ro/La
55
SLE in pregnancy worsens T/F
F
56
You should avoid oestrogen containing OCPs in SLE
T
57
Risks of SLE in pregnancy
Higher risk of premature delivery, feral loss and perinatal mortality. Can give AZA
58
Drug induced SLE In whoabs
Older, uncommon in black ppl, F>M, HLADR4, slow acetylatorsUncommon to have Ro/LaMay be associated with antihistone, antiphopholipid, pANCA (minor), dsDNA (antiATNF)
59
Drug induced SLE often has renal and CNS involvement T/F
F
60
Antihistone abs in drug induced lupus - which drugs
Procainamide, hydralazine, chlorpromazine, quinidine (>90%)Minocycline, propylthiouracil, statins(
61
SCLE - drug induced has anti-histone abs T/F
F
62
In drug induced SCLE, anti SSA will disappear after the drug is ceased in most ppl T/F
T 75% will disappear
63
If C1q is present in DLE DIF what does this mean
Risk of eventual systemic disease
64
3 key histological features of lupus
1. Liquefaction degeneration of basal cell layer2. Degenerative changes of connective tissue - Hyalinisation, oedema, fibrinoid changes3. Patchy dermal infiltrate with a few plasma cells + histiocytes around appendages which may be atrophic
65
What is Kikuchi-Fujimoto disease
histiocytic necrotising lymphadenitis,. Idiopathic systemic inflammatory disease, typically affects cervical lymph nodes. skin changes in 30%. Associated with SLE, HHV6, Parvovirus, EBV, dengue, Yersinia

Chapter 51 connective tissue diseases Melissa (3 decks)

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