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SLE and dermatomyositis Flashcards

1
Q

Malar rash?

A

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

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2
Q

Discoid rash?

A

Erythematous raised patches with adherent keratotic scaling and follicular plugging

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3
Q

Maculopapular or SLE rash of generalized ACLE is present in approximately what percent of patients with SCLE?

A

35 to 60%

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4
Q

ACLE is how many times more common in women than men?

A

8x

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5
Q

DLE characteristics? (SEPTA)

A 
Scale
Erythema
Patulous opening (distended)
Telangiectasia (small dilated blood vessels)
Atrophy
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6
Q

Carpet tack sign?

A

When the adherent scale is lifted from more advanced lesions, keratotic spikes can be seen to project from the undersurface of the scale

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7
Q

What is lupus hair?

A

reversible, nonscarring alopecia
develop during periods of systemic disease activity
may be a form of telogen efluvium as the result of flaring systemic disease

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8
Q

what is the 5 year survival rate for SLE?

A

80-95%

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9
Q

what is the 10 year survival rate for SLE?

A

70 to 90%

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10
Q

what are the ominous prognostic signs in SLE?

A

hypertension
nephritis
systemic vasculitis
CNS disease

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11
Q

percentage of patients with SCLE that develop active SLE, including lupus nephritis?

A

10%

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12
Q

CCLE lesions, typically classic DLE, have also arisen in patients initially presenting with SCLE

A

CCLE lesions, typically classic DLE, have also arisen in patients initially presenting with SCLE

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13
Q

what carcinoma can develop within a longstanding DLE skin lesion?

A

Squamous cell carcinoma

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14
Q

what is the law of lupus?

A

SLE is often falsely accused of causing anything and everything that might happen to a patient subsequent to the diagnosis of SLE.

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15
Q

Define localized DLE.

A

Lesions occur only on the head and neck

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16
Q

Define generalized DLE.

A

Lesions occur above and below the neck

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17
Q

Generalized DLE

A

more commonly associated with underlying SLE
often more recalcitrant to standard therapy(frequently requiring layering of antimalarial and immunosuppressive medications)

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18
Q

How can ACLE be differentiated from true TEN?

A

ACLE occurs on predominantly sun-exposed skin
has a more insiduous onset
The mucosa may or may not be involved as in TEN

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19
Q

How does generalized ACLE present?

A

It presents as a widespread morbiliform or exanthematous eruption often focused over the extensor aspects of the arms and hands
It spares the knuckles

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20
Q

What are the other names for GENERALIZED ACLE?

A

Maculopapular rash of SLE
photosensitive lupus dermatitis
SLE rash

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21
Q

What is the most common form of CCLE?

A

A classic DLE skin lesion

- It is present in 15 to 30% of SLE populations selected in various ways

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22
Q

What are the causes of drug induced systemic lupus erythematosus?

A

HIPMAA

Hydralazine
Isoniazid
Procainamide
Minocycyline
Anti-tumor necrosis factor biologics
Antihyperlipidemic agents
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23
Q

Annular SCLE synonyms

A

Lupus marginatus
symmetric erythema centrifugum
autoimmune annular erythema
lupus erythematosus gyrates repens

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24
Q

Papulosquamous SCLE synonyms

A 
Disseminated DLE
Subacute disseminated LE
Superficial disseminated LE
psoriasiform LE
Pityriasiform LE
maculopapular photensitive LE
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25
Q

What are the nonscarring alopecia?

A

Lupus hair
Telogen effluvium
Alopecia areata

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26
Q

What are the leading causes of disease related death in DM?

A

ILD and assocaited cancers

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27
Q

What percentage of DM patients have an associated malignancy?

A

10-20%

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28
Q

What are the biomarkers for myositis?

A

Creatine kinase
aldolase
LDH

  • however, as the duration of myositis increases, they become insensitive markers of myositis
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29
Q

What are the more sensitive tests for active myositis?

A

EMG and MRI

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30
Q

Describe the Raynaud phenomenon

A

is a medical condition in which the spasm of small arteries causes episodes of reduced blood flow to end arterioles.

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31
Q

What test is specific for myocardial damage?

A

Cardiac troponin I testing.

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32
Q

What percentage of patients with DM are actually minimally photosensitive?

A

60%

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33
Q

What percentage of patients with DM report disease exacerbation after UV exposure?

A

20 percent.

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34
Q

Define calcinosis.

A

The deposition of calcium in the skin, subcutaneous tissue, muscles and visceral organs is known as calcinosis. This condition commonly occurs in the skin, where it is known as calcinosis cutis or cutaneous calcification.

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35
Q

What is dystrophic calcinosis cutis?

A

Dystrophic calcinosis cutis occurs in an area where there is damaged, inflamed, neoplastic or necrotic skin. Tissue damage may be from mechanical, chemical, infectious or other factors. Normal serum calcium and phosphate levels exist. Conditions that can cause dystrophic calcinosis cutis may include:

Trauma
Acne
Varicose veins
Infections
Tumours (pilomatrixoma, cysts, basal cell carcinomas and others)
Connective tissue disease (dermatomyositis, systemic sclerosis, cutaneous lupus erythematosus)
Panniculitis
Inherited diseases of connective tissue (Ehlers-Danlos syndrome, Werner syndrome, Pseudoxanthoma elasticum, Rothmund-Thomson syndrome)
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36
Q

What are the skin examination findings that are SENSITIVE for DM? (MLS)

A

microscopic periungual telangiectasia
lateral digit hyperkeratosis
scalp erythema and dysesthesia ( cutaneous symptom–such as pruritus, burning, tingling, stinging, anesthesia, hypoesthesia, tickling, crawling, cold sensation, or even pain–without a primary cutaneous condition in a well-defined location that is often caused by nerve trauma, impingement, or irritation.)

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37
Q

What are the more specific skin findings in DM? (ROGG)

A

“red on white” patches
ovoid palatal patch
grossly visible periungual telangiectasias
Gottron papules

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38
Q

What is the prevalence of arthralgias in patients with DM?

A

30 to 40% of patients

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39
Q

What is the classic triad of EMG findings of myositis?

A
  1. small amplitude, short duration, polyphasic motor unit potentials
  2. fibrillations and postive sharp waves
  3. complex repetitive discharges
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40
Q

Bohan and Peter’s diagnostic criteria

A
  1. Symmetrical weakness of the limb girdle muscles and anterior neck flexors
  2. Muscle biopsy evidence of necrosis of myofibers, phagocytosis, regeneration with basophils, large vesicular sarcolemmal nuclei and prominent nucleoli, atrophy in a perifascicular distribution, variation in fiber size and an inflammatory exudate, often perivascular
  3. Elevation in serum of skelatal muscle enzymes, particularly the CK and often aldolase, AST, ALT and LDH
  4. Electromyographic triad of small amplitude, short duration, polyphasic motor unit potentials; fibrillations and positive sharp waves; and complex repetitive charges
  5. Any one of the characteristic dermatologic features of the rash of DM
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41
Q

What reflects active disease in DM?

A

Panniculitis

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42
Q

Increased risk of ILD autoantibody in DM?

A

Anti-tRNA synthetase

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43
Q

Antisynthetase syndrome? (FAM RIM)

A 
ILD (interstitial lung disease)
fever
arthritis
myositis
mechanic's hands
Raynaud phenomenon
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44
Q

Frequency of Jo1 in adult dermatomyositis?

A

20 %

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45
Q

Hall mark cutaneous disease? antibody?

A

Anti-Mi-2

46
Q

Good prognosis and response to therapy antibody?

A

Anti-Mi-2

47
Q

Relapses common antibody?

A

Anti-Mi-2

48
Q

High CK values (>5000 U/L) antibody?

A

Anti-Mi-2

49
Q

Rare ILD and Cancer antibody?

A

Anti-Mi-2

50
Q

most severe muscle involvement antibody?

A

Anti-Mi-2

51
Q

Good therapeutic response antibody?

A

Anti-Mi-2

52
Q

Increased cancer risk antibody?

A

Anti-Tif1-gamma

53
Q

severe cutaneous disease antibody?

A

anti-Tif1-gamma

54
Q

Red on white skin lesions autoantibody?

A

Anti-Tif1-gamma

55
Q

Ovoid palatal patch autoantibody?

A

Anti-Tif1-gamma

56
Q

Low ILD risk autoantibody?

A

Anti-tif1-gamma

57
Q

Low CK values (200-2000 U/L)

A

Anti-TIF1-gamma

58
Q

high ILD risk autoantibody?

A

anti-MDA5

59
Q

skin ulceration autoantibody?

A

anti-MDA5

60
Q

red palmar papules autoantibody?

A

anti-MDA5

61
Q

alopecia autoantibody?

A

anti-MDA5

62
Q

gingival pain autoantibody?

A

anti-MDA5

63
Q

arthritis autoantibody?

A

anti-MDA5

64
Q

isolated high aldolase auto antibody?

A

Anti-MDA5

65
Q

Increased cancer risk in adults autoantibody?

A

anti-NXP2

66
Q

increased risk of calcinosis?

A

Anti-NXP2

67
Q

peripheral edema autoantibody?

A

AntiNXP2

68
Q

Myalgia, autoantibody?

A

AntiNXP2?

69
Q

severe dyshpagia autoantibody?

A

AntiNXP2

70
Q

distal weakness autoantibody?

A

antiNXP2

71
Q

Skin disease onset before myositis autoantibody?

A

Anti SAE

72
Q

May have severe skin disease autoantibody?

A

AntiSAE

73
Q

Dysphagia autoantibody?

A

Anti-SAE

74
Q

Positive finding of antiphospholipid antibodies based on?

A
  1. abnormal serum level of IgG or Igm anticardiolipin antibodies
  2. a positive test result for lupus anticoagulant
  3. a false positive serologic test for syphilis known to be positive for at least 6 months and confirmed by Treponema pallidum immbolization or fluorsecent treponemal antibody absorption test
75
Q

What is the most common form of CCLE?

A

Classic DLE lesion.

76
Q

What is the percentage that classic DLE presents in SLE populations?

A

15-30%

77
Q

DLE occurs most common in individuals between?

A

20-40 years of age

78
Q

DLE has a female to male ratio of?

A

3:2 to 3:1

79
Q

What is the first line for SLE?

A

Topical glucorticoids and topical calcineurin inhibitors and intralesonal TA (2.5 -10 mg/cc)

80
Q

What is the 2nd line for SLE?

A

Hydroxychloroquine 6.5 mg/kg/day based on ideal body weight

Chloroquine 3-3.5 mg/kg/day based on ideal body weight

81
Q

HOw much prednisone in lupus?

A

short course for 2-16 weeks

5-60 mg/day

82
Q

Third line for lupus?

A

Mycophenolate mofetil
Azathioprine
Methotrexate

83
Q

what are the drugs that should be avoided in lupus because they are potentially photosensitizing?

A

Hydrochlorthiazide
Tetracycline
griseofulvin
Piroxicam

84
Q

What is a photosensitive form of CLE that presents as a reticulated array of macules and or papules on the upper chest and back?

A

Reticulated erythematosus mucinosis

85
Q

What is the lupus band test?

A

refer to the examination of nonlesional skin biopsies for the presence of this bandlike array of immunoreactants at the dermal-epidermal junction

86
Q

What are observed in the lesional and nonlesional skin of patients with LE?

A

IgG, IgA, IgM
C3, C4, C1q, properdin, factor B
MAC C5b-C9

87
Q

Nonlesional lupus band test?

A

When 3 or more immunoreactants are present at the DEJ the diagnostic specificity for SLE appears to be very high

88
Q

What is the percentage for anti-Ro/SSa in SCLE?

A

70 to 90%

89
Q

What is the perentage of anti-La in SCLE?

A

30-50%

90
Q

What is the percentage of rheumatoid factor in SCLE?

A

33%

91
Q

Lupus erythematosus tumidus?

A

succulent, edematous, urticaria-like plaques with little surface change
appears to be the most photosensitive subtype of cutaneous lupus
demonstrates good response to antimalarials
tend to resolve completely without either sccarring or atrophy

92
Q

What antibody is the association for chilblain LE?

A

anti-Ro/SSA antibodies

93
Q

What percentage of Chilblain LE develop SLE?

A

20%

94
Q

What is LE profundus?

A

LE panniculitis + DLE lesions.

95
Q

Describe the lesions of LE panniculitis/LE profundus

A

Overlying skin often becomes attached to the subcutaneous nodules and is drawn inward to produce deep, saucerized depression.

96
Q

What percentage of Mucosal DLE occurs in patients with CCLE?

A

25%

97
Q

What is the most frequent affected in mucosal discoid lupus?

A

Oral mucosa
lesions begin as painless erythematous paches tthat evolve to chronic plaques
Chronic buccal mucosal plaques are sharply marginated and have irregularly scalloped, white borders with radiating white striae and telangiectasia
The surfaces of these plaques overlying the palatal mucosa often have a honey comb appearance

98
Q

Overlapping features of hypertrophic LE and lichen planus have been describer under the rubric?

A

Lupus planus

99
Q

Lupus erythematosus hypertrophicus et profundus

A

appear to represent a rare form of hypertrophic DL

100
Q

Patient with hypertrophic DLE probably do not have a greater risk for developing SLE than do patients with classic DLE lesions

A

Patient with hypertrophic DLE probably do not have a greater risk for developing SLE than do patients with classic DLE lesions

101
Q

How does classic DLE lesions begin?

A

They begin as red-purple macules, papules or small plaques and rapidly develop a hyperkeratotic surface

102
Q

What is the percentage of patients of DM with an associated malignancy?

A

10-20%

103
Q

What is often elevated in anti-MDA5 patients with DM?

A

serum ferritin (>500 mg/dL)

104
Q

What is a sensitive indicator of myositis and correlates with creatine kinase levels?

A

Muscle edema

105
Q

How do you describe myalgias in DM?

A

Soreness or muscle tightness or burning, but muscles are NOT tender to palpation

106
Q

What are arthralgias in DM?

A 
they are common 
reported in 30-40% of patients
involve small joints of the hands (wrists, MCP and PIP)
shoulders 
elbows
ankles
107
Q

What are the most common ECG abnormalities in DM?

A

ST-T segment changes and condunction abnormalities

108
Q

What is a useful biomarker in detecting subclinical cardiac muscle involvement?

A

Cardiac Troponin I

109
Q

High risk for cardiac involvement antibody?

A

anti-MDA5

110
Q

What are the risk factors for increased risk of malignancy in DM?

A 
increasing age
male gender
cutaneous necrosis
dysphagia
rapid onset of myositis
111
Q

What does muscle and skin biopsies of DM show?

A

CD3 + T cells
Plasmacytoiod dendritic cells
macrophagesB cells

112
Q

How does parenchymal cell injury manifest in the skin and muscle? (DM)

A

By interface dermatitis with keratinocyte injury and in muscle by atrophy, degeneration and regeneration of muscle fibers typically in a perifascicular distribution.

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