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Inflammatory Connective Tissue Disease > RANDOM > Flashcards

RANDOM Flashcards

1
Q

Inflammatory myopathy often overlap with other CTDs typically

A

MCTD
SLE
Sjogren
SSc

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2
Q

Predominant bacteria that cause infectious myopathy in children

A

Staph
Strep

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3
Q

MC implicated viral agent of viral myositis

A

Influenza B

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4
Q

viral myositis: onset of symptoms and resolution

A

3 days after onset of fever and respi symptoms; up to 1 month

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5
Q

Gene affected in muscular dystrophies

A

Dystrophin gene

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6
Q

MC age of muscular dystrophies

A

2-3y

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7
Q

Pseudohypertrophy of calves is seen in

A

muscular dystrophies

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8
Q

Painless prox muscle weakness is seen in

A

Hyperthyroid myopathy

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9
Q

3 MC malig associated with IIM

A

Leukemia
CNS tumors
Lymphoma

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10
Q

Classic histopath of skin lesions in JDM

A

Vacuolar interface dermatitis with dermal mucin deposition

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11
Q

Mainstay of tx of JDM

A

High dose CS + steroid-sparing agents

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12
Q

Most widely used treatment for JDM

A

CS

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13
Q

Most widely used steroid-sparing agent for JDM

A

MTX

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14
Q

MTX dose/route for JDM

A

15-20mg/bsa/wk, SC

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15
Q

Treatment for steroid resistant JDM

A

IVIg

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16
Q

Options for JDM with persistent skin rash despite resolution of muscle disease (2)

A

IVIg, MMF

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17
Q

Option for JDM intolerant to MTX

A

Cyclosporine, 3-5mkday

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18
Q

Option for severe or refractory JDM

A

CYC

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19
Q

Option for JDM with skin-predominant disease without significant muscle involvement

A

HCQ 5mkday max 400/day

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20
Q

T/F JDM patients should be encouraged to exercise even with active disease

A

T

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21
Q

Cure for JDM lipodistrophy

A

None

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22
Q

JDM: Reliable test to monitor disease activity

A

None

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23
Q

Muscle strength tests for JDM

A

MMT
CMAS
MMT8

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24
Q

Frequency of disease damage assessment in JDM

A

At least annual

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25
Q

Course of JDM

A

1/3 monocyclic
3-30% polycyclic
30-60% chronic

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26
Q

Predicts chronic course in JDM

A

Higher disease activity score at diagnosis

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27
Q

Skin vs MSK: More rapid improvement

A

MSK

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28
Q

JDM Predictors of longer time to remission

A

Rash 3 months after dx
Abn nailfold capillary and rash at 6m

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29
Q

Causes of death in JDM

A

GI, pulmonary, unresponsive myositis, CV, infection

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30
Q

MC damaged organ system in JDM

A

Skin

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31
Q

HRQoL domain that is most impaired in JDM

A

Physical well-being

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32
Q

Median onset of lipodystrophy in JDM

A

4y after dx

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33
Q

T/F skin biopsy of locaclized and systemic sclerosis are indistinguishable

A

T

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34
Q

MC affected organs in JSSc

A

Vascular
Cutaneous
GI
Pulmo
MSK

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35
Q

JSSc gender predilection

A

F

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36
Q

A diagnosis of JSSc should be suspected in the ff

A

1) Very young child with RP
2) Older child with moderate to severe RP with associated nailfold cap abnormalities, digital ulcers, and Ab profile of SSc

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37
Q

2007 Classification criteria for JSSc

A

Major (Required): Skin induration/thickening proximal to the MCP or MTP joints

Minor (2 required): Scleroderma-specific organ involvement

*Age of onset must be <16y
Page 381, Table 27.1

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38
Q

ACR/EULAR classification criteria for SSc (Adult)

A

Page 381, Table 27.2
Definite SSc: Total score of ≥9

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39
Q

ACR/EULAR classification criterion for SSc (Adult) with automatic score of 9

A

Skin thickening of both hands extending proximal to the MCPs

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40
Q

Clinical subtypes of SSc

A

Diffuse cutaneous
Limited cutaneous
Overlap syndrome

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41
Q

Antibody associations: Diffuse cutaneous SSc

A

Anti Scl-70

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42
Q

Antibody associations: Limited cutaneous SSc

A

Anti centromere

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43
Q

Antibody associations: Overlap syndrome SSc

A

Anti PM-Scl

44
Q

Clinical subtype of SSc: Rapidly progressive skin thickening

A

Diffuse

45
Q

Clinical subtypes of SSc: Early visceral disease

A

Diffuse

46
Q

Clinical subtypes of SSc: Restricted, nonprogressive skin thickening

A

Limited

47
Q

Clinical subtypes of SSc: Late visceral disease

A

Limited

48
Q

Clinical subtypes of SSc: CREST syndrome

A

Limited

49
Q

CREST syndrome

A

Calcinosis, RP, esophageal dysmotility, sclerodactyly, telangiectasia

50
Q

Clinical subtypes of SSc: Features of SSc + another CTD

A

Overlap

51
Q

Commonly overlaps with JSSc

A

JDM

52
Q

Adult vs Juvenile SSc: Overlap

A

JSSc

53
Q

MC clinical feature of SSc

A

RP

54
Q

Adult vs Juvenile SSc: Better survival rates

A

Juvenile

55
Q

3 phases of Scleroderma skin lesions

A

Edematous
Sclerosis (or induration)
Atrophy

56
Q

Predilection of JSSc edematous phase

A

Distal extremities

57
Q

Onset of sclerosis phase of JSSc

A

Several weeks to months after edematous phase

58
Q

Fibrotic phase of JSSc is paticularly noticeable where

A

Dorsum of fingers and hands (acrosclerosis)

59
Q

TYpical scleroderma facies due to sclerotic skin changes

A

Pinched nose
Thin pursed lips
Small mouth with diminished aperture
Prominent teeth
Expressionless appearance

60
Q

mRSS scores how many areas of the body

A

17

61
Q

mRSS scores areas of the body as

A

0-3, none, mild, mod, severe

62
Q

Calculated from mRSS and assist in predicting internal organ involvement in those with dcSSc

A

Skin thickness progression rate (STPR)

63
Q

Telangiectasia of SSc is most prominent in these areas

A

Face and upper ext

64
Q

Color changes of RP

A

pallor-cyanosis-erythema

65
Q

Findings in NFC of SSc

A

Dilated (>2x normal width) and tortuous THEN dropout (absence of NFC) and arborization of capillaries

66
Q

Normal finger-to-palm measurement

A

0 (able to touch fingertip to palmar crease)

67
Q

FTP is a component of

A

Medsger severity scale of SSc

68
Q

Typical myopathy in SSc affects what muscle group

A

Proximal and typically symmetrical

69
Q

How to measure FTP

A

Place a pen marking at the distal palmar crease at the base of the middle finger; ask patient to fully flex the middle finger; measure distance from distal palmar crease to tip of patient’s finger in full flexion

70
Q

Children with dcSSC and this organ system involvement are at particular risk for severe cardiac involvement

A

Myositis

71
Q

T/F GI manifestation may precede skin involvement in JSSc

A

T, in 42-74%

72
Q

T/F SSc typically progreses through the GIT from top to bottom

A

T

73
Q

PFTs in JSSc that are particularly concerning

A

Decreased FVC
Decreased DLCO

74
Q

Major cause of Scleroderma related mortality in children with SSc

A

Cardiac involvement

75
Q

Major cause of Scleroderma related mortality in adults with SSc

A

Lung involvement

76
Q

PAthognomonic pathological finding in the heart of patients with SSc

A

Mosaic patchy distribution of myocardial fibrosis

77
Q

Recommended baseline cardiac exams for JSSc

A

ECG and echo

78
Q

Mild renal dysfxn commonly occurs in JSSC due to

A

Vasculopathy rather than GN

79
Q

ADult vs juvenile SSc: SCleroderma renal crisis

A

Adult

80
Q

Medical emergency in SSc characterized by accelerated arterial htn, renal insufficiency, microangiopathic hemolytic anemia, and thrombocytopenia

A

SRC

81
Q

Baseline renal test recommended for SSC patients

A

UA
Spot UPCr
BP

82
Q

T/F Neuro involvement is rare in SSc

A

T

83
Q

Subtype of localized scleroderma that is most likely to resemble JSSc

A

Pansclerotic morphea, most sever type of LS

84
Q

Important diff between JSSc and pansclerotic morphea

A

1) In pansclerotic morphea, although fingers and hands may be swollen from venous congestion from surrounding sclerosis, thickness of the skin and skin changes are rarely found distal to the MCPs and PIPs. In contrast, JSSc usually starts with skin changes and thickness of the distal ext and proceeds proximally.
2) RP, NFC changes, and digital ulcerations are not significant in pansclerotic morphea

85
Q

Characteristic findings of the skin in eosiniphilic fasciitis

A

1) Peau d’orange
2) Groove sign due to deep facial involvement

86
Q

Treatment for scleroderma renal crisis

A

ACEis (catopril or enalapril) given immediately

87
Q

PRevention of SRC

A

Immediate lowering of BP in SSc and malignant htn
Avoid any sudden changes in plasma volume, particularly marked reductions

88
Q

Organ specific treatment for Systemic Sclerosis

A

Table 27.8, Page 395

89
Q

SSc, first-line treatment: RP

A

CCB (nifedipine, amlodipine)

90
Q

SSc, first-line treatment: Digital ulcers

A

PDE-5 inhibitors (sildenafil, tadalafil)

91
Q

SSc, first-line treatment: Pulmonary htn

A

PDE-5 inhibitors, endothelin receptor antagonists

92
Q

SSc, first-line treatment: ILD

A

CYC, MMF, CS

93
Q

SSc, first-line treatment: Skin

A

MTX, MMF, CS

94
Q

SSc, first-line treatment: MSK

A

NSAID, HCQ, MTX, CS

95
Q

SSc, treatment for very severe disease: RP

A

Prostacyclins (iloprost)
Sympathectomy (botulinum toxin)
Fat grafting

96
Q

SSc, treatment for very severe disease: Digital ulcers

A

Prostacyclins

97
Q

SSc, treatment for very severe disease: ILD

A

Hematopoietic stem cell transplantation

98
Q

SSc, treatment for very severe disease: Skin

A

CYC, rituximab

99
Q

SSc, treatment of GI manifestations: Tooth decay

A

Fluoride tx every 3 months

100
Q

SSc, treatment of GI manifestations: Dysphagia

A

Soft foods, small portions, adeq fluids, low sugar, gingival mucosa grafting

101
Q

SSc, treatment of GI manifestations: Esophageal dysmotility, reflux, constriction

A

Metoclopramide, erythromycin 3-5mkday
Wt loss
Small freq meals
Avoid eating 3h prior to bed
Raise had of bed 6 inches
Avoid tight clothes, heavy lifting, bending
PPI, H2 blockers

102
Q

SSc, treatment of GI manifestations: Watermelon stomach (GAVE), fructose intolerance

A

Metoclopramide, erythromycin, ocreotide

103
Q

SSc, treatment of GI manifestations: Small int bacterial overgrowth

A

FODMAP diet

104
Q

Adult vs Juvenile SSc: More favorable prognosis

A

Juvenile

105
Q

MC cause of death

A

Cardiac (including PAH) and respi failure

Inflammatory Connective Tissue Disease (8 decks)

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