Lupus Erythematosus Flashcards
What are the 3 major types of Lupus erythematosus?
Acute (ACLE), subacute (SCLE), and chronic (CCLE)
Are the 3 types of cutaneous lupus mutally exclusive?
No, every form of cutaneous lupus can be seen in a patient –> they can have more than one type
What level of ANA do you need to be considered +?
In adults <65 y/o: 1:160 In adults >65 y/o: 1:320
Are there any cutaneous lupus findings that are not associated with systemic lupus?
No, every form of cutaneous lupus may be seen in the setting of SLE or as an isolated cutaneous dz. -However, the degree of association with SLE (systemic dz) varies by cutaneous type.
What antibody do you use to monitor lupus associated nephritis?
dsDNA.
What percent of the population have + ANA’s at 1:40, 1:80, and 1:160?
30%, 13% and 5% respectively
What autoantibody is + in neuropsychiatric LE but only 7% of normal LE?
rRNP (ribosomal P protein)
How prevalent is a + ANA in lupus?
99%
How prevalent is ssDNA in LE and what is its significance when positive?
Denature DNA, + in 70% of LE cases. Possible risk for SLE in DLE patients. Also seen in RA, MCTD, localized scleroderma, systemic sclerosis, Sjogren’s syndrome.
Prevalence of Ro (SS-A) autoantibodies in LE?
LE = 50%, SCLE (75-90%), neonatal LE/congenital heart block (99%), SCLE-SjS overlap
Prevalence and significance of cardiolipin autoantibody in LE
50% in LE, cardiolipin is a negatively charged phospholipid. Important for antiphospholipid syndrome. Recurrent spontaeous abortions, thrombocytopenia, and hypercoagulable state in SLE.
Cutaneous manifestations of antiphospholipid syndrome?
Livedo reticularis, leg ulcers, acral infarction/ulceration, hemorrhagic cutaneous necrosis. -Similar associations in primary antiphospholipid antibody syndrome, clinical associations strongest with IgG class of anticardiolipin
Prevalence of Histone autoantibodies and significance in LE?
40% of LE, important in drug-induced SLE, RA
Prevalence and importance of B2 (beta 2) glycoprotein I antibody in LE?
25% in LE, it’s an important cofactor for cardiolipin in cardiolipin autoantibody assays. It confers a relatively high risk of thrombosis in SLE and primary antiphospholipid antibody syndrome.
Clinical presentation of acute cutaneous lupus erythematosus (ACLE)?
Non-scarring, facial/malar erythema, photodistributed exanthematous to urticarial eruption (favors: upper chest, extensor arms, dorsal hands w/ sparing of knuckles) and can be widespread –> to include non-photodistrubted areas.
Clinical presentation of subacute cutaneous lupus erythematosus (SCLE)?
Non-scarring, annular, or papulosquamous eruption in photodistributed sites - favor upper trunk and upper outer arms >> lateral neck, forearms, hands . Often SPARES the mid-face/malar region - long term pigmentary changes common after resolution.
What percentage of those with SCLE meet criteria for dx of SLE and what is the phenotype of their disease?
50% meet the criteria. Rarely have serious systemic sequelae, arthralgias are the most common.
What autoantibody is common in SCLE?
Anti-Ro/SSA is positive in 70%
Clinical presentation of discoid lupus or chronic cutaneous lupus erythematosus? (CCLE)
Localized, ofthen on the head and neck. Can have widespread or lesions beyond the head and neck. There is also an unusual varient, hypertropic DLE which have thick scale overlying or at periphery of lesions. -CCLE can involve mucosal surfaces, palms and soles - occur in both UV and non-UV exposed sites early lesions are inflamed, indurated plaques with surrounding scale → well-established lesions have follicular plugging (clinically hyperkeratosis), atrophy, scarring, and alopecia - examine concha bowl for follicular plugging and scarring
What percentage of patients with DLE go on to have SLE?
Those with head and neck involvment (5-10% –> SLE) Widespread involvement of the extremities and trunk (~20% –> SLE)
What are the 3 other types of chronic cutaneous LE? (CCLE)
Tumid LE, lupus panniculitis, Chilblain LE
Clinical presentation of tumid LE?
Onset is delayed by 1-2 weeks of inciting stimulus (UVR) b/c pathology is deeper -Most common on the face and upper trunk (erythema, induration, central clearing) - ABSENCE of follicular plugging, scarring, and atrophy.
What percentage of those with tumid LE go on to SLE?
<1% → SLE
Presentation of lupus panniculitis?
Absence of scale with eventual lipoatrophy favoring the face, upper outer arms, upper trunks, breasts, thighs - Presence of discoid lesions + lipoatrophy = lupus profundus