61 - Lupus Erythematosus Flashcards
The term LE-specific relates to those lesions displaying a/an
Interface dermatitis
Most frequent clinical manifestation of LE
Joint inflammation
Second most frequent clinical manifestation of LE
Skin disease
ACLE
- Localized ACLE (malar rash, butterfly rash)
- Generalized ACLE (lupus maculopapular rash, SLE rash, photosensitive lupus dermatitis)
a. TEN-like ACLE
SCLE
- Annular SCLE
- Papulosquamous SCLE
*Drug-induced
CCLE
- Classic DCLE
a. Localized DLE
b. Generalized DLE - Hypertrophic/verrucous DLE
- Lupus profundus/lupus panniculitis
- Mucosal DLE
- Oral DLE
- Conjunctival DLE
- Lupus tumidus (urticarial plaque phase of LE)
- Chillblain LE (chillblain lupus, perniotic lupus)
- Lichenoid DLE (LE/lichen planus overlap, lupus planus)
Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds
Malar rash
Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions
Discoid rash
Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation
Photosensitivity
Oral ulcers: oral or nasopharyngeal ulceration, usually (painful/painless), observed by a physician
Painless
Serositis criteria
a. Pleuritis - convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion
or
b. Pericarditis - documented by electrocardiogram or rub or evidence of pericardial effusion
Renal disorder criteria
a. Persistent proteinuria - >0.5 g/day or greater than 3+ if quantitation not performed
or
b. Cellular casts - may be red cell, hemoglobin, granular, tubular, or mixed
Neurologic disorder criteria
a. Seizures - in the absence of offending drugs or known metabolic derangements (eg, uremia, ketoacidosis, or electrolyte imbalance)
or
b. Psychosis - in the absence of offending drugs or known metabolic derangements (eg, uremia, ketoacidosis, or electrolyte imbalance)
Hematologic disorder criteria
a. Hemolytic anemia - with reticulocytosis
or
b. Leukopenia - < 4000 μL total on 2 or more occasions
or
c. Lymphopenia - < 1500/μL on 2 or more occasions
or
d. Thrombocytopenia - < 100,000 μL in the absence of offending drugs
Immunologic disorder criteria
a. Anti-DNA - antibody to native DNA in abnormal titer
or
b. Anti-Smith antigen - presence of antibody to Smith nuclear antigen
or
c. Positive finding of antiphospholipid antibodies based on (1) an abnormal serum level of igG or igM anticardiolipin antibodies, (2) a positive test result for lupus anticoagulant using a standard method, (3) a false-positive serologic test for syphilis known to be positive for at least 6 months and confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test
Antinuclear antibody criteria
An abnormal titer of antinuclear antibody by immunofluorescence of an equivalent assay at any point in time and in the absence of drugs known to be associated with “drug-induced lupus” syndrome
1982 Revised Criteria for Classification of SLE: a person shall be said to have SLE if any _____ or more of the _____ criteria present
4
11
_____ SCLE patients are somewhat older at disease onset
Drug-induced
Most common form of CCLE
Classic DLE
Approximately _____% of patients presenting with isolated localized DLE subsequently develop SLE
5
Most upregulated gene pathway identified in microarray studies in SLE patients
Type 1 IFN
Produce large amounts of type 1 IFN in response to DNA and RNA stimulation through toll-like receptors 7 and 9
Plasmacytoid dendritic cells
Probably the most important environmental factor in the induction phase of SLE
UV radiation
Y/N: Smokers are at a greater risk of developing SLE than are nonsmoker and former smokers
Yes
Patients with treatment-resistance CLE were much more likely to
Smoke
Patients with LE-specific skin disease who smoke are less responsive to
Anti-malarial treatment
Seroconversion to _____ virus among patients with SLE is nearly universal
Epstein-Barr virus
Y/N: The designations acute, subacute, and chronic, in regard to CLE, refer to how long individual lesions have been present.
No - Refer to the pace and severity of any associated SLE and are not necessarily related to how long individual lesions have been present
Most commonly imputed drug classes causing drug-induced lupus
Antacids
Antifungals
Biologics
Chemotherapeutic
Localized ACLE has commonly been referred to as
Classic butterfly rash or
Malar rash of SLE
Characteristically spared in malar rash
Nasolabial folds
Generalized ACLE presents as a widespread morbilliform or exanthematous eruption often focused over the extensor aspects of the arms and hands and characteristically sparing the
Knuckles
Perivascular nail fold erythema and telangiectasia are considerably more common and occur in more exaggerated forms of
Dermatomyositis
Systemic sclerosis
An extremely acute form of ACLE is rarely seen that can simulate
Toxic epidermal necrolysis
ACLE can be differentiated from true TEN because it
Occurs on predominantly sun-exposed skin
Has a more insidious onset
A finding of circulating autoantibodies to the _____ strongly supports a diagnosis of SCLE
Ro/SS-A ribonucleoprotein particle
Erythema multiforme-like lesions occurring in patients with SLE in the presence of La/SS-B autoantibodies
Rowell syndrome
When the face is involved with SCLE, it is most often the _____, with sparing of the _____
Lateral face
Central, malar regions
A consistent clinical difference is that DLE lesions are characteristically _____, whereas SCLE lesions are not
Indurated
Risk factors for the development of SLE in a patient presenting with SCLE lesions
Papulosquamous type of SCLE
Leukopenia
High titer of ANA (>1:640)
anti-dsDNA antibodies
When the adherent scale is lifted from more advanced DLE lesions, keratotic spikes similar in appearance to _____ can be seen to project from the undersurface of the scale
Carpet tacks
May represent telogen effluvium occurring as the result of flaring systemic disease
Lupus hair
Y/N: LE skin disease activity can be precipitated by any form of cutaneous trauma
Yes - Koebner phenomenom or isomorphic effect
Perforation of the nasal septum is more often associated with _____ than DLE
SLE
Kaposi-Irrgang disease
LE profundus/LE panniculitis
Chillblain LE appears to be associated with _____ antibodies
anti-Ro/SS-A
Helps to distinguish chillblain LE from idiopathic chillblains
Persistence of lesions beyond the cold months
Positive ANA
Presence of one of the other ACR criteria for SLE
Y/N: The characteristic epidermal histologic changes of LE-specific skin disease are only minimally expressed, if at all in lupus erythematosus tumidus
Yes
Most photosensitive subtype of cutaneous lupus
Lupus erythematosus tumidus
Y/N: Lupus erythematosus tumidus typically demonstrates a poor response to antimalarials
No - good response
Y/N: Lupus erythematosus tumidus lesions tend to resolve completely without either scarring or atrophy
Yes
The presence of LE-nonspecific skin changes, especially when seen in conjunction with LE-specific rashes, corresponds with
Higher systemic disease activity
The laboratory markers for SCLE are the presence of _____ (70% - 90%) and, less commonly, _____ (30% - 50%) autoantibodies
Anti-Ro/SS-A
Anti-La/SS-B
Approximately _____% of the patients with SCLE develop active SLE
10
Evidence suggests that overlap occurs between SCLE and
Sjogren syndrome
First line treatment for LE-specific skin disease
Topical glucocorticoids, topical calcineurin inhibitor
Intralesional triamcinolone acetonide
Second line treatment for LE-specific skin disease
Hydroxychloroquine
Chloroquine
Quinacine
If monotherapy fails, add quinacrine to either hydroxychloroquine or chloroquine
Short course treatment (2-16 weeks only) for LE-specific skin disease
Prednisone
Thalidomide
Third line treatment for LE-specific skin disease
Azathioprine
Mycophenolate mofetil
Methotrexate
Maximum dose of hydroxychloroquine
6.5 mg/kg/day based on ideal body weight
Maximum dose of chloroquine
3-4 mg /kg/day
Approximately _____ are required to reach equilibrium blood levels of hydroxychloroquine
6 weeks
If no response is seen after _____ of hydroxychloroquine, quinacrine hydrochloride 100 mg/day can be added
8-12 weeks
Antimalarial that does not cause retinopathy
Quinacrine
Doses of _____ may need to be adjusted for patients with decreased renal or hepatic function
Quinacrine
Chloroquine is generally felt to be more efficacious than hydroxychloroquine in treating CLE, perhaps because of the
Earlier therapeutic responds that might occur as a result of the shorter time period required to reach steady-state blood levels
Should not be used simultaneously because of the enhanced risk of retinal toxicity
Hydroxychloroquine
Chloroquine
(Hydroxychloroquine/Chloroquine) may be more retinotoxic than (Hydroxychloroquine/Chloroquine)
Chloroquine
Hydroxychloroquine
Y/N: Modern hydroxychloroquine regimen can be used safely in children and in women who are pregnant
Yes
Antimalarial associated with higher incidence of side effects
Quinacrine
Commonly produces a yellow discoloration of the entire skin and sclera in fair-skinned individuals, which is completely reversible when the dose of the drug is reduced or discontinued altogether
Quinacrine
Can produce significant hemolysis in patients with G6PD deficiency
Quinacrine
Can produce bone marrow suppression, including aplastic anemia, although this effect is exceedingly rare with the current dosage regimens
Each of the aminoquinolone antimalarials
Significant dose-related and/or methemoglobinemia can result from the use of
Dapsone
Sensory neuropathy is another toxicity associated with _____, and 25% to 75% of patients with CLE develop peripheral neuropathy while taking this drug
Thalidomide
Thromboembolism is a serious adverse event that may occur in patients with a preexisting hypercoagulable state
Thalidomide
Thalidomide analog
US FDA approved for the treatment of multiple myeloma
Lenalidomide
Safety data to date indicates a much lower rate of peripheral neuropathy with (thalidomide/lenalidomide) compared to (thalidomide/lenalidomide); however it has a similar rate of thromboembolism (especially when combined with glucocorticoids) and leukopenia compared to (thalidomide/lenalidomide)
Lenolidomide
Thalidomide
Thalidomide
Because steroid-induced bone loss occurs most rapidly in the first _____ of use, all patients who do not have contraindications should begin agents to prevent osteoporosis with the initiation of steroid therapy
6 months
(Prednisone/Prednisolone) rather than (Prednisone/Prednisolone) should be used in patients who have significant underlying liver disease, because (Prednisone/Prednisolone) requires hydroxylation in the liver to become biologically active
Prednisolone
Prednisone
Prednisone
Any amount of prednisone given as a single oral dose in the morning has (more/less) adrenal-suppressing activity than the same amount given in divided doses throughout the day
Less
Any given amount of prednisone, taken in divided doses, has a (greater/lesser) LE-suppressing activity than does the same amount of drug given as a single morning dose
Greater