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M&M Test 4 > B6-023 Dermatomyositis/Scleroderma > Flashcards

B6-023 Dermatomyositis/Scleroderma Flashcards

(67 cards)

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1
Q

proximal muscle weakness with skin involvement

A

dermatomyositis

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2
Q

proximal muscle weakness without skin involvement

A

polymyositis

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3
Q

anti-Jo 1 antibody

A

polymyositis

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4
Q

occurs in older adults with distal extremity involvement
responds poorly to treatment

A

inclusion body myositis

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5
Q

have 1 or all of the following findings:
myositis, ILD, raynaud’s, inflammatory arthropathy, mechanic’s hand

A

antisynthetase syndrome (Anti-Jo-1)

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6
Q

early complement deposition with vascular involvement

A

dermatomyositis

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7
Q

B cells and CD 4+ T cells in perifasicular and perivascular infiltration

A

dermatomyositis

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8
Q

endomysial CD8+ T cells around non-necrotic fibers

A

polymyositis/IBM

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9
Q

vacuoles with rim of eosinophilic granules

A

IBM

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10
Q

used to treat dysphagia caused by dermatomyositis

A

IVIG

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11
Q

1st line steroid sparing agents used with high dose steroid for dermatomyositis [2]

A

methotrexate
azathioprine

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12
Q

used for ILD due to dermatomyositis or resistant dermatomyositis [2]

A

rituximab
mycophenolate

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13
Q

treatment of dermatomyositis

A
  1. prednisone- high dose, taper
  2. immunosuppressant
  3. long term management and reassessment
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14
Q

what antibodies have high risk of malignancy associated with dermatomyositis?

A

anti-p155/140
anti-MJ

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15
Q

only skin and soft tissue involvement
no Raynaud’s
kids > adults

A

localized scleroderma

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16
Q

puffiness of hands
Raynauds
sclerodactly
salt and pepper vitiligo
reduced oral aperture

A

scleroderma

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17
Q

triad of pathophysiology of scleroderma

A
  1. autoantibodies
  2. obliterative vasculopathy
  3. fibrosis
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18
Q

main effector cytokine that drives myofibroblast differentiation

A

TGF-b

**causes fibrosis

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19
Q

late vascular manifestation of limited cutaneous scleroderma

A

pulmonary hypertension

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20
Q

all manifestations of diffuse cutaneous scleroderma occur […] in the timeline

A

early

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21
Q

antibody strongly associated with limited cutaneous systemic sclerosis

A

Anti-centromere Ab

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22
Q

CREST syndrome patients have

A

limited cutaneous systemic sclerosis

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23
Q

increased risk of isolated PAH

A

limited cutaneous systemic sclerosis

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24
Q

what antibody is associated with diffuse cutaneous scleroderma?

A

anti-topoisomerase-1 Ab (Scl-70)

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25
what type of scleroderma is more likely to have severe interstitial lung disease?
diffuse cutaneous scleroderma
26
what antibody is associated with skin thickening that might predate Raynaud's?
anti-RNA polymerase III
27
which antibody in scleroderma is most strongly associated with cancer?
anti-RNA polymerase III
28
Anti-SSA (Ro) and SSB (La) antibodies
Sjogren's syndrome
29
dry eyes and mouth arthralgia fatigue vaginal dryness upper airway disease
Sjogren's syndrome
30
risk of lymphoma is elevated in [...]
Sjogren's
31
[...] must be positive in mixed connective tissue disease
anti-RNP
32
skin thickening raynaud's positive ANA/ACA
systemic sclerosis
33
limited vs diffuse cutaneous is defined based on
extent of skin involvement
34
systemic sclerosis involving only distal arms and legs, and face
limited
35
endomysial inflammation CD 8+ lymphocytes
polymyositis
36
myocyte vacuoles on trichrome stain
IBM
37
erythematous or violaceous papules over the MCP/IP joints may have white scaling
gottron's papules
38
first line treatment for dermatomyositis
high dose prednisone tapered over months coupled with methotrexate or azathioprine
39
used in combination with prednisone/azathioprine for treatment of dermatomyositis if dysphagia is present
IVIG
40
used for resistant dermatomyositis or in cases with ILD
rituximab
41
what antibody is limited cutaneous systemic sclerosis associated with?
anti-centromere (ACA)
42
what antibody is most commonly associated with polymyositis?
Anti-Jo-1
43
antibodies associated with diffuse scleroderma [2]
anti-Scl-70 anti-RNA polymerase III
44
perimysial inflammation with atrophy CD 4+ t cells
dermatomyositis
45
photodistributed facial erythema and edema of eyelids associated with dermatomyositis
heliotrope rash
46
thickening, cracking, irregular marks on hands due to hyperkeratinosis of digital skin associated with dermatomyositis
mechanic's hands
47
[polymyositis/dermatomyositis] increased risk of malignancy
dermatomyositis
48
violaceous rash on the upper back with areas of hyper/hypo pigmentation associated with dermatomyositis
shawl and face rash
49
erythematous papules with white scale over the IP and MCP joints of both hands associated with dermatomyositis
gottron's papules
50
what would you expect to see on skin biopsy of dermatomyositis?
interface dermatitis **inflammation of the DEJ
51
perifasicular inflammation and atrophy
dermatomyositis
52
prominent vasculopathy including gastrointestinal vasculitis leading to GI bleed/perforation subtype of dermatomyositis
juvenille dermatomyositis
53
absent skin involvement anti-jo-1 antibody
polymyositis
54
older adults distal extremity involvement poor Rx response
IBM
55
why doesn't raynaud's typically cause necrosis of the fingers?
thermoregulation is done by A-V shunts in the skin nutritional flow is provided by a separate capillary network that lies deeper
56
what causes primary Raynaud's?
idiopathic
57
what causes secondary raynaud's?
secondary to mixed connective tissue disease
58
is digital ulceration seen in primary or secondary Raynaud's?
secondary
59
treatment of Raynaud's
calcium channel blockers
60
Raynaud's occurring in younger patients with symmetric involvement and normal labs
primary
61
nailfold capillary changes would be associated with [...] Raynaud's
secondary
62
anti-centromere ab is associated with
limited cutaneous systemic sclerosis CREST syndrome
63
anti-topoisomerase-1 ab (Scl-70-ab) is associated with
diffuse cutaneous systemic sclerosis
64
what antibody in systemic sclerosis is most heavily associated with increased risk of malignancy?
anti-RNA polymerase III
65
what antibody in systemic sclerosis is most heavily associated with rapidly progressive skin thickening that may predate the onset of Raynaud's?
anti-RNA polymerase III
66
systemic sclerosis patients with anti-RNA poly III antibodies require vigilant [...] monitoring
ambulatory BP **higher risk of Scleroderma Renal Crisis
67
Anti-RNP must be positive to diagnose
mixed connective tissue disease

M&M Test 4 (14 decks)

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