Lupus Flashcards
DLE
who
peak onset
Associations
F>M
4th decade in F, later in males
C2 deficiency in females associated.
14-26% raynauds, 30% anaemia, leukopenia, thrombocytopenia
26% false +ve syphilis, 17% RF +ve
1/3 alopecia
20% elevated ESR, increase in serum globulins
Disseminated DLE
Who gets it
What does it look like
Women, who usually smoke
Persistent, resistant to therapy
May look like papulosquamous SCLE but scarring occurs
reticulate telangiectasia
LE gyratum repents: associated with underlying malignancy, negative lupus band.
Swindling of fingers+ hyperextension of distal phalanges
Factors associated with onset of DLE
Trauma, stress, UV exposure, Drugs , seasonal (summer> winter, premenstrual
Autoantibodies in DLE
5-60% +ve ANA (homogenous> speckled)
More common in long disease, extensive, with raynauds, joint pains, chilblains
0.-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
DLE
Nails
Eyes
Mucous membranes
Nails: subungual hyperkeratosis, redblue nail plate, longitudinal striae
Mucous 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
Chillblain lupus
Who
Progression to SLE
Abs
Smokers, can occur years post DLE, and persist with tx when DLE remits.
Usually precipitated by pregnancy
Some 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.
Treatment of DLE
General measures
- psyche, camouflage, photoprotection
Topical
- potent topical steroids CS, often under occlusion
-IL CS, CO2 laser, PDL for telangiectasia
Oral
- nifedipine for raynauds
Pred for severe 0.5mg/kg, taper over 6/52
HCQ 200mg bd then reduce, most respond by 6/.52
stop smoking
Can use thalidomide 100mg/day, cyclophosphamide
DDx of DLE
Morphea, LS, LP Jessners/REM Lupus vulgaris Rosacea NLD Chilblain PMLE Blooms
Prognosis of DLE
SCC in 3.3%, scalp, ears, lip, nose >20 years Scarring 57% scarring alopecia 35% Premenstrual flare 20% 50% complete remission
Associations of DLE that make it more likely to be difficult to treat
raynauds, scalp, chilblain like lesions
Lupus panniculitis
% with DLE clinically and histologically
How does it heal
3-5% DLE clinically, 30% histologically
Heals with anetoderma
Histology of lupus panniculitis
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
Lupus panniculitis - associations
Trauma, biopsy, EMG, monoclonal gammopathy, thrombocytopenic purport
Usual sites of lupus panniculitis
Face, proximal extremities
Concomitant SLE in lupus panniculitis
10-20%
What do you have to histologically exclude from lupus pannicullitis
Subcutaneous panniculitis like T cell lymphoma
T/F immune deposits occur in DEJ in lupus panniculitis
T
ABs in Rowell’s
Speckled ANA, RF, anti La (SSB)
LE hypertrophicus et profundus
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
% of DLE that progresses to SLE
Rook 5% (22% disseminated, 1.2% head and neck)
BJD 16% progression within 3 years of Dx
Bologna 5-10%
Females
Does SCLE scar
Not usually, pigment changes
Drugs and SCLE
Griseofulvin, Antihistamines, Terbinafine, calcium channel blockers, HCT, NSAIDS, PPIs, antitnf.
% of SCLE with anti-ro
70% more likely if on face
ARA criteria (old for SLE)
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
Most specific autoantibodies for SLE
antidsDNA and antiSmAbs
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
Tx SLE
Photoprotection
o UVB is biggest problem (midday sun is high in UVB)
Topicals
o Topicals or IL steroids, tacrolimus +/- keratolytics
Systemics
o 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
Treatment of lupus panniculitis
clobetasol under occlusion, HCQ, ILCS, oral thalidomide
Tx of SCLE
photoprotection
Topical or IL CS, tacrolimus
HCQ, oral CS or methylpred
acitretin, isotretinoin, capstone, MTX, thalidomide, IFNalpha, MMF
NEW SLE criteria 4/17
At least 1 clinical + 1 immunological Clinical: ACLE or SCLE Chronic cutaneous lupus Non scarring alopecia Oral or nasal ulcers Joint disease serositis Renal Neurological Haemolytic anaemia Leukopenia or lymphopenia Thrombocytopenia
Immunological:
ANA, antidsdna, anti-SM, antiphopholipid, low C3,C4, low CH50, direct cooks in absence of haemolytic anaemia
Antiphospholipids
Lupus anticoagulant
anticardiolipin
B2 glycoprotein
Antiphospholipid syndrome associated with
Libman sacks endocarditis, MI, valvular heart disease, a vascular necrosis
Thrombophlebitis, purport, lively, leg ulcers, gangrene, subungual splinter haemorrhages
HLA for annular phenotype of SCLE
HLA-DR3 (B8, other)
SCLE autoantibodies
60% homogenous ANA, 80% antiRo SSA, 16% anticardiolipin Ab
Histo of SCLE cf to DLE
More epidermal atrophy,k less hyperkeratosis, less BM thickening, less follicular plugging, and inflammatory infiltration
More basal vascular change, dermal oedema and mucin
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)
Neonatal lupus % with skin lesions Precipitated by.. Scarring? Residual lesions?
50%
UV, phototherapy for jaundice
Improves in 1st few months, resolves without scarring by 12/12.
May get residual telangiectasia, dyspigmentation ,atrophy, atrophic scarring.
Neonatal lupus RIsk of : Heart block Pacing Dilated cardiomyopathy Thrombocytopenia Mortality
Heart block 60% Pacing 50% Dilated cardiomyopathy 20% Thrombocytopenia 20% (transient) Mortality 20%
Other non haem or cardiac association with neonatal lupus
Liver: neonatal hepatitis, hepatomegaly + splenomegaly.
Ix of neonatal lupus
FBC, elfts - thrombocytopenia and liver involvement
ECG, echo
Cardiology review
If steroids given first 16/40, no conduction defects
Harm review - may need prod 2mg/kg/day
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
Precipitate of SLE
UV
Sprouts, seeds, alfalfa, heavy metals (cadmium, mercury, gold)
Infection
Premenstrual flare in 20%
Drugs eg hydralazine, procainamide, TNFalpha, penicillamine, isoniazid, minocytcline.
Poor prognostic factors of SLE
Renal involvement more organs, males, Asians+ black, arthritis, antiphospholipid, thrombocytopenia, haemolytic anaemia and CNS involvement.
Good prognostic factors for SLE
CS, early Dx, decrease stress and drugs such as sulphonamides, control of infection with Abs
SLE usual initial presentation
% with rash
58% articular
14% cutaneous
80% will have a rash
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 fingers
Hyperkeratosis, ragged cuticles
Hair: 50% alopecia: diffuse or scarring, 30% coarse, dry, fragile hair.
Significance of livedo outer arms in SLE
More frequently in CNS
Risk of membranous lupus nephropathy in SLE
8%
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
Bullous lupus
histo
subepidermal blister with Neutrophils and microabscesses. Linear deposition of IgA, IgG and IgM, C3 Type 1 (type VII collagen), 2 (other antigens) 3 (epidermal antigens)
Where does bullous LE occur
Face, neck, upper trunk
1/3 have mouth lesions
Associations with bullous lupus
glomerulonephritis
Drugs eg hydralazine, IFN alpha
T/F SLE is worse in childhood
T
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
SLE in pregnancy worsens T/F
F
You should avoid oestrogen containing OCPs in SLE
T
Risks of SLE in pregnancy
Higher risk of premature delivery, feral loss and perinatal mortality. Can give AZA
Drug induced SLE
In who
abs
Older, uncommon in black ppl, F>M, HLADR4, slow acetylators
Uncommon to have Ro/La
May be associated with antihistone, antiphopholipid, pANCA (minor), dsDNA (antiATNF)
Drug induced SLE often has renal and CNS involvement T/F
F
Antihistone abs in drug induced lupus - which drugs
Procainamide, hydralazine, chlorpromazine, quinidine (>90%)
Minocycline, propylthiouracil, statins(
SCLE - drug induced has anti-histone abs T/F
F
In drug induced SCLE, anti SSA will disappear after the drug is ceased in most ppl T/F
T 75% will disappear
If C1q is present in DLE DIF what does this mean
Risk of eventual systemic disease
3 key histological features of lupus
- Liquefaction degeneration of basal cell layer
- Degenerative changes of connective tissue - Hyalinisation, oedema, fibrinoid changes
- Patchy dermal infiltrate with a few plasma cells + histiocytes around appendages which may be atrophic
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
