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FD9 > 62 - Dermatomyositis > Flashcards

62 - Dermatomyositis Flashcards

1
Q

Have the characteristic rash of dermatomyositis but no clinical signs or symptoms of myositis

A

Clinically amyopathic dermatomyositis

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2
Q

Have the characteristic DM rash but no clinical signs or symptoms of myositis

A

Clinically amyopathic dermatomyositis (CADM)

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3
Q

_____% of patients have CADM

A

20

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4
Q

If not CADM, then the rest of patients with DM have

A

Classic dermatomyositis

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5
Q

CADM can be further subclassified into

A

Hypomyopathic CM

Amyopathic DM

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6
Q

Hypomyopathic DM is classified if one of these test results is abnormal

A
  1. Magnetic resonance imaging
  2. Electromyography
  3. Muscle biopsy
  4. Laboratory studies of muscle enzymes
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7
Q

CADM in which all the test results are normal

A

Amyopathic DM

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8
Q

Y/N: It has become clear that muscle histology and clinical findings can be defined that are specific to DM

A

No - cannot be defined

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9
Q

Three major criteria in Sontheimer’s proposed diagnostic criteria for cutaneous dermatomyositis

A
  1. Heliotrope sign
  2. Gottron papules
  3. Gottron sign
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10
Q

Violaceous erythema over the eyelids

A

Heliotrope sign

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11
Q

Papules overlying the MCP and IP joints

A

Gottron papules

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12
Q

Erythema overlying the knees, elbows, IP joints, olecranon processes, patellae, and medial malleoli

A

Gottron sign

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13
Q

Sontheimer suggested that a diagnosis of CADM could be made if _____ major criteria were present or _____ major and _____ minor criteria were present in addition to biopsy of at least one region showing histopathologic changes consistent with DM

A

2

1, 2

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14
Q

CADM is an important group to recognize because these patients appear to harbor the same risks for _____ as their classic counterparts

A

Systemic disease

Malignancy

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15
Q

DM has a bimodal distribution in the age of disease onset, occurring at two peaks, at _____ years and _____ years of life

A

5 to 14

45 to 64

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16
Q

DM triggers

A

Substantial ultraviolet exposure
Strenuous activity (for the patient with concurrent myositis)
Recent malignancy

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17
Q

There is often significant pruritus associated with affected skin, particularly on the _____, which may also be described as a “tightness” or burning or with other dysesthetic qualities such as crawling or tingling

A

Scalp

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18
Q

Characteristic cutaneous feature of affected skin in DM

A

Violaceous patches and plaques, varying from a bright pink to a deep violet color

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19
Q

Trunk involvement seen on the posterior neck, upper back, and shoulders, which may extend to the posterior upper arms

A

Shawl sign

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20
Q

Confluent violaceous erythema on the sun-exposed areas of the lower anterior neck and anterior chest

A

V-neck sign

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21
Q

Generally, (juvenile/adult) DM more characteristically displays atrophy and poikiloderma in classic areas of Gottron sign

A

Juvenile

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22
Q

Violaceous erythema ad poikiloderma on the lateral hips and lateral thighs

A

Holster sign

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23
Q

Hyperkeratosis and fissuring along the medial thumb and lateral second and third digits

A

Mechanic’s hands

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24
Q

Mechanic’s hands is a cutaneous clue to the possible presence of

A

Interstitial lung disease

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25
Q

Proximal nailfold involvement can be highly suggestive of

A

DM

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26
Q

Ulceration may be present in 30% of patients and often affects the skin over the _____ although it can be found anywhere

A

Extensor joint surfaces

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27
Q

Cutaneous ulceration in DM warrants concern for the presence of

A

Anti-MDA5 antibodies or

Malignancy

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28
Q

Ulcers have been correlated with the presence of _____, but this is likely through their association with anti-MDA5 antibodies

A

Interstitial lung disease

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29
Q

Symmetric violaceous patch across the midline of the hard palate

A

Ovoid palatal patch

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30
Q

Ovoid palatal patch is most frequently observed in the subset of patients with DM with

A

Anti-transcriptional intermediary factor 1gamma (TIF1-gamma)

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31
Q

Ovoid palatal patch could be confused with oral findings of discoid lupus or lichen planus, but their consistent localization to the _____ may aid in the diagnosis of DM

A

Center of the hard palate

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32
Q

Although _____ is often an important sign of activity, it can often be a sign of damage as well

A

Erythema

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33
Q

Distinctive and pathognomonic pattern composed of reticulated, sometimes atrophic white macules adjacent to erythema or telangiectasias

A

Red on white

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34
Q

The thin skin along the _____ is a frequent place to visualize the “red-on-white” pattern

A

Bitemporal hairline

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35
Q

This morphology is a very useful diagnostic tool because it does not seem to be associated with other connective tissue diseases

A

Red on white

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36
Q

Atrophy, hypopigmentation, hyperpigmentation, and telangiectasias

A

Poikiloderma

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37
Q

_____, as opposed to the “red-on-white”, is a late manifestation and is not diagnostically specific

A

Poikiloderma

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38
Q

Typically a late manifestation in the skin, subcutaneous tissue, fascia, or muscle and typically affects the trunk proximal extremities, or areas of previous disease activity

A

Calcinosis

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39
Q

Calcinosis occurs more rapidly after disease onset in (juvenile/adult) versus (juvenile/adult) DM

A

Juvenile

Adult

40
Q

Calcinosis is most frequent on the _____ in DM, in contrast to systemic sclerosis in which _____ calcinosis is most frequent

A

Extremities

Digital

41
Q

In both juvenile and adult DM, the presence of _____ antibodies is associated with an increased risk of calcinosis

A

Anti-nuclear matrix protein 2 (NXP-2)

42
Q

Calcinosis is also commonly seen in the _____ subset, which as associated with known vasculopathy

A

Anti-MDA5

43
Q

Reflects active disease in DM, typically affecting the buttocks, trunk, and proximal extremities
May progress to calcinosis or lipoatrophy

A

Panniculitis

44
Q

Histopathology shows a _____ panniculitus but may have features of lupus panniculitis with lipomembranous changes or with septal thickening as in deep morphea

A

Lobular

45
Q

Patients with _____ antibodies have a higher risk of alopecia, which commonly is severe and occurs early in the disease

A

Anti-MDA5

46
Q

Has been recently found to be associated with anti-NXP2 antibodies and may predict more severe muscle disease

A

Subcutaneous edema

47
Q

Most common pulmonary manifestation in DM and is a leading cause of morbidity and mortality

A

Interstitial lung disease

48
Q

Pulmonary function tests show a

A

Restrictive disease pattern with a decreased forced vital capacity or a decreased diffusion capacity of carbon monoxide

49
Q

Most common radiographic and histologic pattern in DM

A

Nonspecific interstitial pneumonia

50
Q

Rare manifestation in DM

PFT revealing a disproportionately low diffusion capacity of carbon monoxide compared with a relatively normal FVC

A

Pulmonary arterial hypertension

51
Q

Gold standard for the diagnosis of pulmonary arterial hypertension

A

Right heart catheterization

52
Q

Myositis in DM typically presents as

A

Symmetrical proximal muscle weakness

53
Q

Described as soreness or muscle tightness or burning, but muscles are not tender to palpation

A

Myalgia

54
Q

Patients may note a hoarse or raspy voice (dysphonia) from _____ involvement

A

Cricoarytenoid muscle

55
Q

May occur because of weak pharyngeal musculature

A

Dysphagia

56
Q

Y/N: There is a high correlation between dysphagia and weakness of the anterior neck muscles

A

Yes

57
Q

Distal muscle weakness in the hands, manifesting as difficulty opening jars or holding onto objects, more typically occurs late in disease or in patients with _____ antibodies

A

Anti-NXP2

58
Q

Arthralgias are common in DM, reported in _____% of patients

A

30 to 40

59
Q

Gastrointestinal involvement is an uncommon manifestation in juvenile DM, reported in _____% of juvenile patients with DM

A

4

60
Q

Y/N: Cardiac involvement in DM is usually subclinical

A

Yes

61
Q

May be a useful biomarker in detecting subclinical cardiac muscle involvement

A

Cardiac troponin I

62
Q

DM is associated with malignancy in _____% of cases

A

10 to 20

63
Q

Malignancies tend to occur within the first _____ of disease onset

A

1 to 2 years

64
Q

Major antibody associated with malignancy

A

Anti-TIF1-gamma

65
Q

DM is primarily an immune-mediated disorder with molecular and histologic evidence supporting a role for _____ immunity

A

Both innate and adaptive

66
Q

Y/N: Myositis is not a requirement for the diagnosis of DM

A

Yes

67
Q

A patient presenting with alopecia, cutaneous or mucosal ulceration, palmar erythematous papules, severe arthralgia or arthritis, and shortness of breath should trigger the clinician to consider

A

Anti-MDA5 DM

68
Q

Patients with mechanic’s hands, arthritis, Raynaud phenomenon, and lung symptoms are at high risk of having

A

Antisynthetase disease

69
Q

Patients with extreme myalgia, peripheral edema, and distal weakness should be ruled out for DM specifically with

A

Anti-NXP2 antibodies

70
Q

Myositis-specific autoantibodies associated with DM

A 
TIF1-gamma
NXP2
MDA5
SAE
Mi-2
Jo-1
Other antisynthetase antibodies (PL-7, PL-12, EJ, OJ, SRP)
71
Q

Early in the course of the disease, _____ are reasonably sensitive biomarkers of muscle inflammation. However, mid to late in the course of myositis, their sensitivity decreases

A

Serum muscle enzymes

Electromyographic studies

72
Q

Serum muscle enzymes

A 
Creatine kinase
Aldolase
Lactate dehydrogenase
Aspartate aminotransferase
Alanine aminotransferase
73
Q

To increase the sensitivity of detecting myositis in laboratory testing, the clinician should

A

Evaluate creatine kinase, aldolase, and LDH as a group

74
Q

All muscle enzymes can be elevated after strenuous activity; in questionable cases, the enzyme levels can be rechecked after

A

10-14 days following the activity

75
Q

Can be elevated with liver disease or hemolysis of the blood sample

A

Aldolase

AST and ALT

76
Q

Will be elevated with hepatic injury but will not be elevated in myositis
Helpful addition to laboratory testing for myositis if hepatic sources are suspected

A

Gamma-glutamyl transferas

77
Q

Serum ferritin is often highly elevated (>500 mg/dL) in _____ patients

A

Anti-MDA5

78
Q

Sensitive indicator of myositis on MRI and correlates with creatine kinase levels

A

Muscle edema

79
Q

If a muscle biopsy is necessary to confirm the diagnosis of DM, it has the highest yielded if performed within _____ of beginning any immunomodulatory therapy

A

2 weeks

80
Q

Most common source of persistent disease skin activity

A

Skin over muscles or other organs

81
Q

Y/N: Cutaneous DM often has discordant treatment response with the muscle disease, with recalcitrant skin disease continuing years after the muscle disease is in remission

A

Yes

82
Q

In addition to a complete history and physical examination, routine age-appropriate cancer screening studies (_____), and relevant screening blood work (_____) as well as a urinalysis are indicated

A

Colonoscopy, mammogram, prostate examination

Complete blood count, renal and liver function tests

83
Q

The manual muscle testing for a subset of eight muscle groups may be performed, which is a validated muscle test in which eight major muscle groups (_____) that are the highest yield in idiopathic inflammatory myopathies

A 
Neck flexors
Deltoids
Biceps
Wrist extensors
Gluteus maximus
Gluteus medius
Quadriceps
Ankle dorsiflexors
84
Q

Serum muscle enzymes are checked _____ as biomarkers or myositis

A

At each visit

85
Q

Found in both cardiac as well as regenerating skeletal muscle, so it is not a specific test, although it could be used as a screening test for cardiac involvement

A

Creatine kinase-MB

86
Q

First line treatment of skin disease in DM

A

Photoprotection
Topical steroids
Hydroxychloroquine or chloroquine
Quinacrine

87
Q

First line treatment of muscle disease in DM

A

Systemic corticosteroids

88
Q

Second line treatment of skin and muscle disease in DM

A

Methotrexate
Mycophenolate mofetil
IVIg
Azathioprine

89
Q

Although photoprotection is a key first step in management, up to _____% of patients with DM are actually minimally photosensitive, and as few as _____% may report disease exacerbation after UV exposure

A

60

20

90
Q

First line treatment for myositis in combination with prednisone

A

Methotrexate

91
Q

In patients with DM with suspected or diagnosed ILD, it is prudent to select a different agent than _____ because of its potential to induce acute pneumonitis and pulmonary fibrosis, thereby complicating the evaluation and management of ILD

A

Methotrexate

92
Q

First-line oral agent when ILD is present

A

Mycophenolate mofetil

93
Q

Most likely the single most effective agent for cutaneous DM

A

Intravenous immunoglobulin

94
Q

Commonly used as maintenance therapy in the treatment of ILD associated with idiopathic inflammatory myopathies, typically after induction with cyclophosphamide

A

Azathioprine

95
Q

Calcinosis remains one of the most formidable therapeutic challenges in the DM. _____ for localized lesions remains the most effective and definitive therapy

A

Surgical excision

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