SLE Flashcards
malar rash vs discoid rash
Malar rash – fixed erythema, flat or raised, over malar eminences, sparing nasolabial folds
Discoid rash – erythematous raised patches with keratotic scaling and follicular plugging; can cause atrophic scarring
malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, and serositis (pleuritic pain or rub) or pericarditis can all be seen with ?
SLE
is ANA specific to SLE?
ANA is positive for most SLE but +ANA is also seen in other diseases
two antibodies most diagnostic for SLE?
anti dsDNA and anti Sm
anti histone indicates?
drug induced SLE
anti SSA (ro) and anti SSB (la)
seen with sjogrens syndrome
anti SCL70 ab?
diffuse systemic sclerosis
anti-centromere ab?
limited scleroderma (CREST syndrome)
Antiphospholipid antibodies (to phospholipid B2 glycoprotein complex) also bind to…
and implies what false test?
cardiolipin and lupus anticoagulant
Interfere with in vitro PTT (lupus anticoagulant)
false + syphilis
SLE peak onset
20-40 yo
F>M
SLE genetics
Multi-gene inheritance and big environmental factor role
Specific HLA-DQ alleles linked to anti-dsDNA/anti-Sm;
Deficiencies in C2, C4, or C1q –> failure to clear immune complexes and apoptotic cells
• GWAS = lymphocyte signaling and IFN responses
SLE environmental triggers
• UV light – induce apoptosis and stimulate IL-1 in keratinocytes
• sex hormones influence immune response.
Kleinfelter’s (XXY) increased incidence in males.
• Drugs – hydralazine, procainamide, D-penicillamine, INH. Reversible disease when drug induced.
in SLE increased cytokines is due to…
treat w what drug?
increased BAFF = B cell survival
belumimab (anti BAFF)
IFN elevated in SLE?
IFN1 alpha
lupus susceptibility model
- Susceptibility genes impair self tolerance allowing external environmental triggers to trigger disease. Poor clearance results in persistence of nuclear antigens –> continued immune response
- Problem: env triggers are common yet SLE is not; predisposing genes are common normal alleles (not mutations or null loci)
treatment options for lupus
- Corticosteroids (high activity) vs NSAIDs
- Anti-malarials for skin and organ involvement
- Cyclophosphamide for renal involvement
common causes of death in lupus
renal failure, intercurrent infections, and coronary artery disease (atherosclerosis)
o Coronary artery disease= steroids accelerate the disease
drug induced lupus
• Causative drugs: hydralazine, procainamide, isoniazid, and D-penicillamine
Symptoms: fever, arthritis, and serositis
• NO renal or CNS involvement
• ANA+ and + anti-histone Abs
• Normal serum complement
• Treatment: stop the drug! The disease will resolve!
neuropsychiatric features of SLE
o headaches, seizures, neuropathy, stroke, chorea, and retinopathy (vasculitis), nerve palsy
o psychosis, depression, organic brain syndrome
renal features of SLE
immune complex mediated
o Urinalysis has proteinuria and hematuria
o Serum creatinine increased with renal insufficiency
o High titers anti-dsDNA associated with active renal disease
o 6 classes of Lupus Nephritis
6 classes of lupus nephritis
1 - Minimal mesangial – IC in mesangium, normal light microscopy
2 - Mesangial proliferative – increased mesangial cells and matrix with granular Ig, complement deposition. Normal gloms
3 - Focal LN - thickened capillary wall (wire loop fibrin deposits); renal insufficiency
5 – Membranous – subepithelial IC –> thickened capillary walls; severe proteinuria and nephrotic syndrome
6 - Advanced sclerosing - >90% gloms sclerosed
musculoskeletal features of SLE
o Arthralgias and or arthritis – very common feature of SLE!
Small joints, symmetric, non erosive synovitis
Nondeforming
Swelling and joint pain out of proportion to PE
o Myopathy - Muscle pain and weakness
o Arthritis
cardiac vegetations/endocarditis
subacute cutaneous lupus erythematous
multiple skin lesions with reddened raised borders, central clearing, and light scale, non scarring, widespread
Sun exposed areas like trunk and extremities but not on the face
Associated with anti-SSA/Ro and HL-DR3 genotype
chronic discoid lupus erythematous
face and scalp with thick scales, atrophy, scarring, edema and erythema
Discoid lupus = coin and round shaped erythematous plaques with adherent scale
• Hyperpigmentation and depressed central scarring, atrophy, and depigmentation; usually face and scalp
• Lesion area shows hyperkeratosis – atrophic epidermis with areas of hyperplasia and large lymphoid infiltrates
• ANA+, dsDNA neg, + lupus band test
skin features of SLE
o Ig/complement at dermal-epidermal junction (line of fluorescence at junction) = not specific but highly suggestive
Lymphocytic infiltration, liquefaction of basal layer in epidermis, edema/perivascular lymphocytes in dermis
o Malar erythema (butterfly face rash) – similar on extremities and trunk; spares naso-labial folds; accentuated by sunlight
o Facial erythema – peeling can also occur
o Chronic discoid lupus erythematosus
o Subacute cutaneous lupus erythematosus
o Ulcerations (oral, vaginal, nasal) o Alopecia (atypical hair loss) o Raynauds phenomena (arterial vasospasm in digits of hands and feet in response to cold or emotional stress) o Vasculitis (cutaneous and small vessel involvement)
o Lupus Band Test
immunoglobulin and complement deposition in non-sun-exposed skin –> horizontal band of granular green immunofluorescence at the D-E junction shows deposits of IgM and c3b
common constitutional features of SLE
fever, fatigue, and weight loss
