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Inflammatory Connective Tissue Disease > JDM and Other Inflammatory Muscle Diseases > Flashcards

JDM and Other Inflammatory Muscle Diseases Flashcards

1
Q

Peak age at onset of JDM

A

~7y

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2
Q

T/F JDM is more common in girls

A

T

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3
Q

Allele found to be potentially a major immunogenetic RF for JDM

A

HLA DRB1*0301

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4
Q

Vaccine reported to be associated with development of JDM within 6 months of flare

A

HPV

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5
Q

Cells that play a key role in the pathogenesis of JDM

A

pDCs

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6
Q

Activity of this cytokine is higher in JDM than healthy individuals

A

IFN-a

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7
Q

Mean duration from symptom onset to diagnosis of JDM

A

3-7months

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8
Q

Descriptors of muscle weakness in JDM

A

Symmetrical, more severely affects limb girdle musculature, anterior neck flexors, and trunk muscles

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9
Q

Physical manifestation (sign) that reflects proximal lower extremity weakness

A

Gower’s sign

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10
Q

Physical manifestation (sign) that reflects gluteal muscle weakness

A

Trendelenburg sign

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11
Q

T/F Arthritis of JDM is nonerosive and non-deforming

A

T

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12
Q

MC manifestations of pediatric JDM

A

Fatigue, muscle weakness, heliotrope rash, gottron papules

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13
Q

T/F Reduced nailfold capillary density is characteristic of JDM

A

T

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14
Q

T/F Normal nailfold capillary density in children with JDM is associated with shorter duration of untreated disease and lower skin disease activity

A

T

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15
Q

T/F Nailfold capillary is associated with skin and muscle disease activity in JDM

A

T

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16
Q

T/F Nailfold capillary can be used to track clinical improvement over time after treatment of JDM

A

T

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17
Q

T/F Calcinosis is rare as a presentation of JDM

A

T, develops during the disease course and is thought to be a scarring response

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18
Q

Consequence of JDM that results from insulin resistance secondary to muscle inflammation and damage

A

Lipoatrophy

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19
Q

T/F Cardiac involvement is common in JDM

A

F, rare

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20
Q

T/F ECG and echo should only be done among patients with JDM who are at risk for cardiac disease

A

F, the SHARE initiative recommends to do these in all patients with JDM at diagnosis

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21
Q

T/F ILD is common in JDM

A

F, rare but one of the most serious complications

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22
Q

T/F PFTs are recommended for all patients at diagnosis of JDM

A

T

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23
Q

T/F ILD in JDM usually presents with unilateral lesion on the upper lobe

A

F, bilateral, lower lobe

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24
Q

T/F In any patient with renal disease and features if JDM, a diagnosis of SLE is more likely

A

T

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25
Q

T/F Most case described as juvenile IIM with renal involvement require additional immunosuppressive agent/s for the renal involvement

A

F, most respond to conventional treatment for IIM

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26
Q

Accounts for 80% of all children with IIM

A

JDM

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27
Q

Alleles identified as protective factors, seen less frequently in Caucasians with JDM

A

HLA DQA1*0202, *0101, and *0102

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28
Q

Non-HLA-associated genes implicated in the pathogenesis of JDM

A

TNF-a and IL-1 receptor antagonist

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29
Q

TNF-a allele associated with a more prolonged disease course (≥36 months) and calcification in JDM, as it synthesizes more TNF-a, which may be associated with persistent immune activation

A

TNF-a-308A

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30
Q

TNF-a allele associated with small-vessel occlusion and increased concentration of circulating TSP-1, which enhances proliferation and migration of vascular smooth muscle cells

A

TNF-a-308A

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31
Q

IL-1 receptor antagonist gene allele identified as a risk factor for juvenile IIM in Caucasians but not in African Americans

A

A1 allele

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32
Q

IL-1 receptor antagonist gene allele identified as a possible risk factor for juvenile IIM in African Americans

A

A3 allele

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33
Q

Environmental factors reported to be related to disease flare in JDM

A

1) Unusual sun exposure
2) NSAID use within the preceding 6 months
3) Anti-htn and psychiatric medications
4) HPV vaccine within 6 months of flare
5) Infection

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34
Q

The main Type 1 interferon-producing cells

A

pDCs

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35
Q

Cytokines implicated in the pathophysiology of JDM

A

1) Type 1 IFN (IFN-a)
2) Type 2 IFN (IFN-γ)

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36
Q

Type 2 IFN is predominantly produced by

A

NK cells and activated T cells

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37
Q

JDM: MC Constitutional manifestation

A

Fatigue

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38
Q

JDM: MC MSK manifestation

A

Muscle weakness

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39
Q

JDM: MC Cutaneous manifestation

A

Heliotrope rash, Gottron papules

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40
Q

Normal nail fold capillary density values

A

7-11 capillaries/mm

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41
Q

Nailfold capillary changes seen in JDM

A

1) Capillary dropout
2) Enlarged and elongated capillaries (giant capillaries)
3) Bushy capillaries
4) Areas of hemorrhage

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42
Q

Calcinosis in JDM tends to be present in what areas

A

Pressure points

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43
Q

Can be detected in the serum of JDM patients early in the disease course regardless of symptoms

A

KL-6

44
Q

Antibodies associated with ILD in JDM

A

MDA5

45
Q

Diagnosis of JDM is confirmed using

A

Bohan and Peter criteria 1975

46
Q

Bohan and Peter Criteria for JDM

A

DEFINITE JDM
Pathognomonic rash (heliotrope/gottron) + 3/4
1. Proximal muscle weakness
2. Elevated muscle nz
3. EMG changes of chronic inflammatory myositis
4. Histopath changes of inflammatory myositis

PROBABLE JDM
Pathognomonic rash + 2/4

47
Q

EULAR/ACR JDM classification score that corresponds to definite IIM

A

≥7.5 w/o muscle biopsy
≥8.7 with muscle biopsy

48
Q

EULAR/ACR JDM classification score that corresponds to definite IIM

A

5.5 to <7.5 w/o muscle biopsy
6.7 to <8.7 with muscle biopsy

49
Q

EULAR/ACR JDM classification score that corresponds to possible IIM

A

<5.3 w/o muscle biopsy
<6.5 with muscle biopsy

50
Q

Distinguishes juvenile from adult IIM

A

Age at onset of symptoms <18

51
Q

T/F of JDM: A muscle biopsy is NOT required for a definite diagnosis if the pathognomonic skin rash is present

A

T

52
Q

T/F Serum muscle enzymes may be normal at presentation even in patients with active JDM

A

T

53
Q

T/F Levels of elevated muscle enzymes at JDM onset correlate with disease outcome

A

F

54
Q

T/F ESR and CRP may be normal in patients with active JDM

A

T

55
Q

Biomarkers proposed to distinguish active disease vs remission in JDM

A

Galectin-9 and CXCL10

56
Q

Group of autoantibodies present specifically in patients with IIM

A

MSA

57
Q

Group of antibodies that occur in patients with other autoimmune conditions that are associated with myositis

A

MAA

58
Q

MSAs

A

1) Anti-TIF-1γ (anti-p155/140)
2) Anti-NXP2 (formerly anti-MJ)
3) Anti-MDA5
4) Anti-Mi-2
5) Anti-SRP
6) Anti-tRNA synthetase
7) Anti-HMGCR
8) Anti-SAE

59
Q

MAAs

A

1) Anti-Ro
2) Anti-RNP
3) Anti-PM-Scl

60
Q

MSAs: Autoantibodies associated with malignancy in ADULTS

A

Anti-TIF-1γ

61
Q

MSAs: Autoantibodies associated with greater muscle weakness in children

A

Anti-TIF-1γ
Anti-NXP2
Anti-SRP

62
Q

MSAs: Autoantibodies associated with younger age at onset

A

Anti-NXP2 (formerly anti-MJ)

63
Q

MSAs: May predict development of ILD

A

Anti-MDA5

64
Q

MSAs: Autoantibodies associated with classical JDM phenotype

A

Anti-Mi-2

65
Q

MSAs: Autoantibodies associated with high ANA titers

A

Anti-Mi-2

66
Q

MSAs: Autoantibodies associated with cardiac abnormalities

A

Anti-SRP

67
Q

MSAs: Autoantibodies associated with chronic continuous disease course

A

Anti-SRP

68
Q

MSAs: Autoantibodies associated with older age at onset

A

Anti-tRNA synthetase

69
Q

MSAs: Autoantibodies associated with more insidious onset of disease

A

Anti-tRNA synthetase

70
Q

MSAs: Autoantibodies associated with only a partial response to immunosuppressive meds

A

Anti-HMGCR

71
Q

MSAs: Autoantibodies associated with disease that seems to be IVIg responsive

A

Anti-HMGCR

72
Q

MC MSA in JDM

A

Anti-TIF-1γ (anti-p155/140)

73
Q

MAAs: Associated with good prognosis in myositis

A

Anti-U1-snRNPs

74
Q

MSAs: Severe cutaneous disease

A

Anti-TIF-1γ (anti-p155/140)

75
Q

MSAs: Predicts poor prognosis

A

Anti-MJ

76
Q

MSAs: Milder muscle disease (low CK levels)

A

Anti-MDA5

77
Q

MSAs: Better prognosis

A

Anti-Mi-2

78
Q

MSAs: Initially myopathic disease in adult DM

A

Anti-SAE

79
Q

MSAs: Very high CK levels

A

Anti-SRP

80
Q

Characteristic BUT nonspecific findings of inflammatory myopathy on EMG

A

1) Increased spontaneous and insertional activity (as evidenced by fibrillation potentials and positive sharp waves)
2) Low amplitude, short duration, polyphasic motor unit action potentials
3) Early recruitment

81
Q

Muscle biopsy should ALWAYS be considered in investigating for JDM when

A

1) Absence of skin rash
2) Atypical presentation

82
Q

Typical muscle biopsy in dermatomyositis

A

1) Perifascicular atrophy
2) Muscle fiber size variation
3) Muscle degeneration and degeneration
4) Centralization of nuclei
5) Vasculopathy
6) Swelling of capillary endothelium
7) Perivascular infiltration of, primarily, pDCs, B cells, CD4+ T cells, and macrophages

83
Q

Electron microscopic findings in dermatomyositis

A

Tubuloreticular inclusions

84
Q

IHC staining findings in JDM

A

1) Increased MHC class I
2) Increased IFN-α/β inducible protein myxovirus resistance A (MxA)

85
Q

IHC staining finding that correlates with disease activity

A

Increased MxA

86
Q

4 domains that make up the scoring tool for histological abnormalities in JDM developed by the Int’l JDM Biopsy Consensus Group

A

1) Inflammatory
2) Vascular
3) Muscle fiber
4) Connective tissue
*Includes overall score of severity using a 10-cm visual analog scale

87
Q

The tool developed by the Int’l JDM Biopsy Consensus Group has been used for what muscles

A

Biceps and quadriceps muscle biopsies

88
Q

T/F The tool developed by the Int’l JDM Biopsy Consensus Group has been evaluated for other IIMs

A

F

89
Q

2 most reliable domains in the tool developed by the Int’l JDM Biopsy Consensus Group that correlate with disease activity

A

1) Inflammatory
2) Muscle fiber

90
Q

T/F Pathological findings in JDM muscle biopsy can be used to determine prognosis

A

T

91
Q

Finding in JDM pretreatment muscle biopsy that predicts a chronic disease course

A

Extensive myopathic changes and central nuclei without basophilia

92
Q

Finding in JDM pretreatment muscle biopsy that predicts a chronic disease course WITH ULCERATION

A

Severe arteriopathic changes
Positive arterial direct IF
Foci of severe capillary loss or endomysial fibrosis
Muscle infarcts

93
Q

T/F Plain radiographs are generally sufficient to determine extent of calcinosis in JDM

A

T

94
Q

Diagnostic modality for JDM that is preferred in cases where MRI or muscle biopsy is not possible

A

Muscle ultrasonography

95
Q

UTZ findings in JDM

A

1) Increased muscle echogenicity
2) Calcification seen as echogenic foci with posterior acoustic shadowing

96
Q

T/F Muscle UTZ in JDM correlates well with disease activity

A

T

97
Q

T/F MRI IV contrast material is NOT necessary to evaluate muscle disease

A

T

98
Q

Appearance of IIM in STIR sequences

A

Increased signal intensity (reflects edema)

99
Q

Imaging modality for JDM that detects muscle atrophy and fatty infiltration in the presence of chronic disease

A

T1-weighted sequences

100
Q

An effective imaging modality to assess whether patient has JDM flare or not when the clinical assessment and lab parameters are equivocal

A

MRI

101
Q

JDM MRI Score (JIS) range

A

0 (normal) to 100 (severe)

102
Q

Ddx for JDM: Characteristic rash of JDM without evidence of muscle involvement

A

Amyopathic Dermatomyositis

103
Q

For amyopathic JDM, characteristic rash should have bee present for at least how long?

A

6 months

104
Q

Ddx for JDM: Patients with classic JDM rash without clinical weakness but who may have evidence of subclinical myositis based on muscle nz, EMG, MRI, or muscle biopsy

A

Hypomyopathic dermatomyositis

105
Q

Only ___% of total JDM cases are myopathic dermatomyositis on further investigations

A

4

106
Q

About ___% of patients with hypomyopathis DM evolve into classic JDM

A

25

107
Q

Ddx for JDM: Muscle weakness in the absence of characteristic skin lesions

A

Juvenile polymyositis

Inflammatory Connective Tissue Disease (8 decks)

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