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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
Q

Most specific autoantibodies for SLE

A

antidsDNA and antiSmAbs

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26
Q

Ix for suspected SLE

A
  • 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
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27
Q

Tx SLE

A

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
Q

Treatment of lupus panniculitis

A

clobetasol under occlusion, HCQ, ILCS, oral thalidomide

29
Q

Tx of SCLE

A

photoprotectionTopical or IL CS, tacrolimusHCQ, oral CS or methylpredacitretin, isotretinoin, capstone, MTX, thalidomide, IFNalpha, MMF

30
Q

NEW SLE criteria 4/17

A

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
Q

Antiphospholipids

A

Lupus anticoagulantanticardiolipinB2 glycoprotein

32
Q

Antiphospholipid syndrome associated with

A

Libman sacks endocarditis, MI, valvular heart disease, a vascular necrosisThrombophlebitis, purport, lively, leg ulcers, gangrene, subungual splinter haemorrhages

33
Q

HLA for annular phenotype of SCLE

A

HLA-DR3 (B8, other)

34
Q

SCLE autoantibodies

A

60% homogenous ANA, 80% antiRo SSA, 16% anticardiolipin Ab

35
Q

Histo of SCLE cf to DLE

A

More epidermal atrophy,k less hyperkeratosis, less BM thickening, less follicular plugging, and inflammatory infiltrationMore basal vascular change, dermal oedema and mucin

36
Q

DIF of SCLE

A

More positive in papulosqumous 88% > angular 29%Overall 60%Granular in cytoplasm, nuclear of basal keratinocytes and in epidermis (unlike other variants)

37
Q

Neonatal lupus % with skin lesionsPrecipitated by..Scarring?Residual lesions?

A

50%UV, phototherapy for jaundiceImproves in 1st few months, resolves without scarring by 12/12. May get residual telangiectasia, dyspigmentation ,atrophy, atrophic scarring.

38
Q

Neonatal lupusRIsk of :Heart blockPacingDilated cardiomyopathyThrombocytopeniaMortality

A

Heart block 60%Pacing 50%Dilated cardiomyopathy 20%Thrombocytopenia 20% (transient)Mortality 20%

39
Q

Other non haem or cardiac association with neonatal lupus

A

Liver: neonatal hepatitis, hepatomegaly + splenomegaly.

40
Q

Ix of neonatal lupus

A

FBC, elfts - thrombocytopenia and liver involvementECG, echoCardiology reviewIf steroids given first 16/40, no conduction defectsHarm review - may need prod 2mg/kg/day

41
Q

Risk of NLE in +ve Ro/SSA

A

2% if mother has no connective its use disease or previous hx of NLE 25% if previously NLE child

42
Q

Precipitate of SLE

A

UVSprouts, seeds, alfalfa, heavy metals (cadmium, mercury, gold)Infection Premenstrual flare in 20%Drugs eg hydralazine, procainamide, TNFalpha, penicillamine, isoniazid, minocytcline.

43
Q

Poor prognostic factors of SLE

A

Renal involvement more organs, males, Asians+ black, arthritis, antiphospholipid, thrombocytopenia, haemolytic anaemia and CNS involvement.

44
Q

Good prognostic factors for SLE

A

CS, early Dx, decrease stress and drugs such as sulphonamides, control of infection with Abs

45
Q

SLE usual initial presentation % with rash

A

58% articular 14% cutaneous 80% will have a rash

46
Q

Clinical skin features of SLE

A

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
Q

Significance of livedo outer arms in SLE

A

More frequently in CNS

48
Q

Risk of membranous lupus nephropathy in SLE

A

8%

49
Q

What is Senear-Usher

A

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
Q

Bullous lupus histo

A

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
Q

Where does bullous LE occur

A

Face, neck, upper trunk1/3 have mouth lesions

52
Q

Associations with bullous lupus

A

glomerulonephritisDrugs eg hydralazine, IFN alpha

53
Q

T/F SLE is worse in childhood

A

T

54
Q

SLE in elderly

A

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
Q

SLE in pregnancy worsens T/F

A

F

56
Q

You should avoid oestrogen containing OCPs in SLE

A

T

57
Q

Risks of SLE in pregnancy

A

Higher risk of premature delivery, feral loss and perinatal mortality. Can give AZA

58
Q

Drug induced SLE In whoabs

A

Older, uncommon in black ppl, F>M, HLADR4, slow acetylatorsUncommon to have Ro/LaMay be associated with antihistone, antiphopholipid, pANCA (minor), dsDNA (antiATNF)

59
Q

Drug induced SLE often has renal and CNS involvement T/F

A

F

60
Q

Antihistone abs in drug induced lupus - which drugs

A

Procainamide, hydralazine, chlorpromazine, quinidine (>90%)Minocycline, propylthiouracil, statins(

61
Q

SCLE - drug induced has anti-histone abs T/F

A

F

62
Q

In drug induced SCLE, anti SSA will disappear after the drug is ceased in most ppl T/F

A

T 75% will disappear

63
Q

If C1q is present in DLE DIF what does this mean

A

Risk of eventual systemic disease

64
Q

3 key histological features of lupus

A
  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
Q

What is Kikuchi-Fujimoto disease

A

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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