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FD9 > 61 - Lupus Erythematosus > Flashcards

61 - Lupus Erythematosus Flashcards

1
Q

The term LE-specific relates to those lesions displaying a/an

A

Interface dermatitis

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2
Q

Most frequent clinical manifestation of LE

A

Joint inflammation

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3
Q

Second most frequent clinical manifestation of LE

A

Skin disease

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4
Q

ACLE

A
  1. Localized ACLE (malar rash, butterfly rash)
  2. Generalized ACLE (lupus maculopapular rash, SLE rash, photosensitive lupus dermatitis)
    a. TEN-like ACLE
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5
Q

SCLE

A
  1. Annular SCLE
  2. Papulosquamous SCLE

*Drug-induced

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6
Q

CCLE

A
  1. Classic DCLE
    a. Localized DLE
    b. Generalized DLE
  2. Hypertrophic/verrucous DLE
  3. Lupus profundus/lupus panniculitis
  4. Mucosal DLE
  5. Oral DLE
  6. Conjunctival DLE
  7. Lupus tumidus (urticarial plaque phase of LE)
  8. Chillblain LE (chillblain lupus, perniotic lupus)
  9. Lichenoid DLE (LE/lichen planus overlap, lupus planus)
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7
Q

Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds

A

Malar rash

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8
Q

Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions

A

Discoid rash

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9
Q

Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation

A

Photosensitivity

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10
Q

Oral ulcers: oral or nasopharyngeal ulceration, usually (painful/painless), observed by a physician

A

Painless

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11
Q

Serositis criteria

A

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

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12
Q

Renal disorder criteria

A

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

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13
Q

Neurologic disorder criteria

A

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)

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14
Q

Hematologic disorder criteria

A

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

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15
Q

Immunologic disorder criteria

A

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

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16
Q

Antinuclear antibody criteria

A

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

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17
Q

1982 Revised Criteria for Classification of SLE: a person shall be said to have SLE if any _____ or more of the _____ criteria present

A

4

11

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18
Q

_____ SCLE patients are somewhat older at disease onset

A

Drug-induced

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19
Q

Most common form of CCLE

A

Classic DLE

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20
Q

Approximately _____% of patients presenting with isolated localized DLE subsequently develop SLE

A

5

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21
Q

Most upregulated gene pathway identified in microarray studies in SLE patients

A

Type 1 IFN

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22
Q

Produce large amounts of type 1 IFN in response to DNA and RNA stimulation through toll-like receptors 7 and 9

A

Plasmacytoid dendritic cells

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23
Q

Probably the most important environmental factor in the induction phase of SLE

A

UV radiation

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24
Q

Y/N: Smokers are at a greater risk of developing SLE than are nonsmoker and former smokers

A

Yes

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25
Q

Patients with treatment-resistance CLE were much more likely to

A

Smoke

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26
Q

Patients with LE-specific skin disease who smoke are less responsive to

A

Anti-malarial treatment

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27
Q

Seroconversion to _____ virus among patients with SLE is nearly universal

A

Epstein-Barr virus

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28
Q

Y/N: The designations acute, subacute, and chronic, in regard to CLE, refer to how long individual lesions have been present.

A

No - Refer to the pace and severity of any associated SLE and are not necessarily related to how long individual lesions have been present

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29
Q

Most commonly imputed drug classes causing drug-induced lupus

A

Antacids
Antifungals
Biologics
Chemotherapeutic

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30
Q

Localized ACLE has commonly been referred to as

A

Classic butterfly rash or

Malar rash of SLE

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31
Q

Characteristically spared in malar rash

A

Nasolabial folds

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32
Q

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

A

Knuckles

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33
Q

Perivascular nail fold erythema and telangiectasia are considerably more common and occur in more exaggerated forms of

A

Dermatomyositis

Systemic sclerosis

34
Q

An extremely acute form of ACLE is rarely seen that can simulate

A

Toxic epidermal necrolysis

35
Q

ACLE can be differentiated from true TEN because it

A

Occurs on predominantly sun-exposed skin

Has a more insidious onset

36
Q

A finding of circulating autoantibodies to the _____ strongly supports a diagnosis of SCLE

A

Ro/SS-A ribonucleoprotein particle

37
Q

Erythema multiforme-like lesions occurring in patients with SLE in the presence of La/SS-B autoantibodies

A

Rowell syndrome

38
Q

When the face is involved with SCLE, it is most often the _____, with sparing of the _____

A

Lateral face

Central, malar regions

39
Q

A consistent clinical difference is that DLE lesions are characteristically _____, whereas SCLE lesions are not

A

Indurated

40
Q

Risk factors for the development of SLE in a patient presenting with SCLE lesions

A

Papulosquamous type of SCLE
Leukopenia
High titer of ANA (>1:640)
anti-dsDNA antibodies

41
Q

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

A

Carpet tacks

42
Q

May represent telogen effluvium occurring as the result of flaring systemic disease

A

Lupus hair

43
Q

Y/N: LE skin disease activity can be precipitated by any form of cutaneous trauma

A

Yes - Koebner phenomenom or isomorphic effect

44
Q

Perforation of the nasal septum is more often associated with _____ than DLE

A

SLE

45
Q

Kaposi-Irrgang disease

A

LE profundus/LE panniculitis

46
Q

Chillblain LE appears to be associated with _____ antibodies

A

anti-Ro/SS-A

47
Q

Helps to distinguish chillblain LE from idiopathic chillblains

A

Persistence of lesions beyond the cold months
Positive ANA
Presence of one of the other ACR criteria for SLE

48
Q

Y/N: The characteristic epidermal histologic changes of LE-specific skin disease are only minimally expressed, if at all in lupus erythematosus tumidus

A

Yes

49
Q

Most photosensitive subtype of cutaneous lupus

A

Lupus erythematosus tumidus

50
Q

Y/N: Lupus erythematosus tumidus typically demonstrates a poor response to antimalarials

A

No - good response

51
Q

Y/N: Lupus erythematosus tumidus lesions tend to resolve completely without either scarring or atrophy

A

Yes

52
Q

The presence of LE-nonspecific skin changes, especially when seen in conjunction with LE-specific rashes, corresponds with

A

Higher systemic disease activity

53
Q

The laboratory markers for SCLE are the presence of _____ (70% - 90%) and, less commonly, _____ (30% - 50%) autoantibodies

A

Anti-Ro/SS-A

Anti-La/SS-B

54
Q

Approximately _____% of the patients with SCLE develop active SLE

A

10

55
Q

Evidence suggests that overlap occurs between SCLE and

A

Sjogren syndrome

56
Q

First line treatment for LE-specific skin disease

A

Topical glucocorticoids, topical calcineurin inhibitor

Intralesional triamcinolone acetonide

57
Q

Second line treatment for LE-specific skin disease

A

Hydroxychloroquine
Chloroquine
Quinacine

If monotherapy fails, add quinacrine to either hydroxychloroquine or chloroquine

58
Q

Short course treatment (2-16 weeks only) for LE-specific skin disease

A

Prednisone

Thalidomide

59
Q

Third line treatment for LE-specific skin disease

A

Azathioprine
Mycophenolate mofetil
Methotrexate

60
Q

Maximum dose of hydroxychloroquine

A

6.5 mg/kg/day based on ideal body weight

61
Q

Maximum dose of chloroquine

A

3-4 mg /kg/day

62
Q

Approximately _____ are required to reach equilibrium blood levels of hydroxychloroquine

A

6 weeks

63
Q

If no response is seen after _____ of hydroxychloroquine, quinacrine hydrochloride 100 mg/day can be added

A

8-12 weeks

64
Q

Antimalarial that does not cause retinopathy

A

Quinacrine

65
Q

Doses of _____ may need to be adjusted for patients with decreased renal or hepatic function

A

Quinacrine

66
Q

Chloroquine is generally felt to be more efficacious than hydroxychloroquine in treating CLE, perhaps because of the

A

Earlier therapeutic responds that might occur as a result of the shorter time period required to reach steady-state blood levels

67
Q

Should not be used simultaneously because of the enhanced risk of retinal toxicity

A

Hydroxychloroquine

Chloroquine

68
Q

(Hydroxychloroquine/Chloroquine) may be more retinotoxic than (Hydroxychloroquine/Chloroquine)

A

Chloroquine

Hydroxychloroquine

69
Q

Y/N: Modern hydroxychloroquine regimen can be used safely in children and in women who are pregnant

A

Yes

70
Q

Antimalarial associated with higher incidence of side effects

A

Quinacrine

71
Q

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

A

Quinacrine

72
Q

Can produce significant hemolysis in patients with G6PD deficiency

A

Quinacrine

73
Q

Can produce bone marrow suppression, including aplastic anemia, although this effect is exceedingly rare with the current dosage regimens

A

Each of the aminoquinolone antimalarials

74
Q

Significant dose-related and/or methemoglobinemia can result from the use of

A

Dapsone

75
Q

Sensory neuropathy is another toxicity associated with _____, and 25% to 75% of patients with CLE develop peripheral neuropathy while taking this drug

A

Thalidomide

76
Q

Thromboembolism is a serious adverse event that may occur in patients with a preexisting hypercoagulable state

A

Thalidomide

77
Q

Thalidomide analog

US FDA approved for the treatment of multiple myeloma

A

Lenalidomide

78
Q

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)

A

Lenolidomide
Thalidomide
Thalidomide

79
Q

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

A

6 months

80
Q

(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

A

Prednisolone
Prednisone
Prednisone

81
Q

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

A

Less

82
Q

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

A

Greater

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