Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

MM Test 2 > B6.023 Dermatomysositis and Scleroderma > Flashcards

B6.023 Dermatomysositis and Scleroderma Flashcards

1
Q

what is interface dermatitis?

A

inflammation at the dermal-epidermal junction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

histo characteristics of interface dermatitis

A

vacuolar change

lymphocytic inflammation that obscures the D/E junction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

differential diagnosis associated with interface dermatitis

A 
SLE
DM 
cutaneous lupus
erythema multiforme
drug eruption
GVHD
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

def of dermatomyositis

A

idiopathic inflammatory myopathy characterized by muscle weakness and rash
complex, chronic, systemic autoimmune disease that can also cause constitutional symptoms, inflammatory arthropathy, calcinosis, and ILD
clinical and serological patterns can vary

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

skin findings associated with DM

A 
gottrons papules
periungal erythema
nailfold capillary changes
heliotrope rash
V sign
Shawl sign
muscle wasting
calcincosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

gottron’s papules

A

interface dermatitis over IP joints

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

nailfold capillary changes

A

palisading array of vessels

cause erythematous appearance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

V-sign and Shawl sign

A

rash in pattern of photo-distribution

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

calcinosis

A

nodular or linear densities appearing under the skin

show up on xray

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

epidemiology of DM

A

2/100,000
2x more common in females
peak incidence age 40-50

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

juvenile DM

A

prominent vasculopathy including GI vasculitis leading to GI bleed or perforation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

DM features

A

proximal muscle and skin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

polymyositis features

A

absent skin involvement

Jo-1-Ab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

inclusion body myositis features

A

older adults
distal extremities involved
poor response to treatment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what is antisynthetase syndrome

A

present in up to 30% of PM and DM patients
constellation of involved organs
antibodies directed against tRNA synthetases (50% are anti-Jo-1)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

pentad of symptoms of antisynthetase syndrome

A 
myositis
ILD**
Raynaud's
inflammatory arthropathy
mechanic's hand
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

histo of DM

A

early complement deposition
vascular changes
later B cell and CD4+ helper T cell infiltrate in a perivascular pattern
perifascicular atrophy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

histo of PM

A

endomysial CD8+ cytotoxic T cells with fewer macrophages around non necrotic fibers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

histo of IBM

A

same as PM +

vacuoles with a rim of eosinophilic granules of varying sizes distributed throughout myocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

pre treatment assessment of DM

A 
overlapping immune conditions (ILD, SSc, SLE)
comorbid conditions (DM2, liver disease, cancer)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

mainstay of DM treatment

A

prednisone
high dose, tapered over months
+ second immunosuppressive agent

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

immunosuppressive agents added to prednisone for DM treatment

A

methotrexate and azathioprine (first line)
IVIg (second line, more transient, used for dysphagia)
rituximab and mycophenolate (for ILD or resistant disease)
hydroxychloroquine (skin and joints)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

what % of PM and DM are associated with malignancy

A

10%

1/3 before, 1/3 concurrently, 1/3 after

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

common cancers associated with PM and DM

A

adenocarcinomas of the cervix, lung, ovaries, pancreas, bladder, and stomach

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

antibodies with a positive association with malignancy

A

antibodies to TIF-1gamma (anti-p155, anti-p155/140)

antibodies to NPX-2 (anti-MJ or anti-p140)

26
Q

antibodies with a negative association with malignancy

A

anti-synthetase antibodies
anti-Mi-2
anti-SRP
myositis associated antibodies (anti-RNP, anti-PM-Scl, anti-Ku)

27
Q

overlap CTD

A

multiple identifiable CTDs

28
Q

mixed CTD

A

patients DO have features of multiple CTDs (systemic sclerosis, myositis, RA, and SLE)
anti-RNP must be positive
various clinical features often present at different times, often separated by years

29
Q

undifferentiated CTD

A

patients have features of CTD (arthralgia, myalgia, fatigue, Raynaud’s, ANA positive) but DO NOT have specific features of any one CTD
many will not progress, but some will

30
Q

what is Raynaud’s

A

an exaggerated response of the digital arterial circulation triggered by cold temperature and emotional stress
exaggeration of a normal response

31
Q

epidemiology of Raynauds

A

present in 3-15% of the normal population
more common in women (3-4:1)
often beings before age 20

32
Q

discuss the pathophysiology of Raynauds

A

vasoconstriction at the level of the digital arteries, precapillary arterioles, and/or cutaneous AV shunts

33
Q

thermoregulation of the skin

A

sympathetic nervous system regulates this process through AV shunts in the skin
nutritional flow to the skin is provided by a separate network of capillaries

34
Q

primary Raynauds

A

very low risk to progress to systemic sclerosis (scleroderma) or another CTD (<1%)

35
Q

therapy for primary Raynauds

A

focused on conservative measures and dihydropyridine Ca channel blockers when necessary
-amlodipine, nifedipine

36
Q

characteristics of primary Raynauds

A
  • younger age <40
  • symmetric
  • absence of necrosis, ulceration, gangrene
  • normal nailfold capillaries
  • negative ANA
  • normal ESR
  • absence of finding to suggest a secondary cause
37
Q

characteristics of secondary Raynauds

A
  • older age (>40)
  • asymmetric attacks
  • severe attacks with ischemia and necrosis
  • abnormal nailfold capillaries
  • positive ANA or other lab studies
  • other systemic disease or causal factors
38
Q

asymmetric Raynaud events or single digit only

A

digital ischemia with vasospasms mimicking RP

39
Q

metabolic disease that could cause Raynauds

A

hypothyroid

40
Q

progression of nailfold capillary changes in scleroderma

A

palisading levels of vessels (normal)
micro hemorrhages
loss of capillary dilated loops
disorganization

41
Q

what is scleroderma

A

heterogenous group of conditions which are linked by having thickened and sclerotic skin lesions
great diversity in manifestations w regard to the extent of skin disease and internal organ involvement

42
Q

2 types of scleroderma classifications

A

localized -skin and soft tissue involvement, no internal organ disease
systemic - have systemic manifestations

43
Q

classes of localized scleroderma

A

morphea
linear scleroderma
scleroderma en coup de sabre

44
Q

what is a morphea plaque

A

large plaque presenting with scaling, induration, and redness on the border
can appear alone or all over body

45
Q

epidemiology of localized scleroderma

A

kids > adults

46
Q

results of localized scleroderma

A

long term morbidity from skin, muscle, and bone atrophy causing growth defects and deformities

47
Q

what is a coup de sabre manifestation

A

sclerotic line of scar tissue down forehead

can get cranial nerve palsies

48
Q

classes of systemic scleroderma

A 
limited cutaneous (lcSSc)
diffuse cutaneous (dcSSc)
sine scleroderma (no skin involvement)
overlap
49
Q

distribution of limited cutaneous systemic scleroderma

A

distal involvement only

50
Q

distribution of diffuse cutaneous systemic scleroderma

A

involvement anywhere on body

51
Q

manifestations of systemic sclerosis

A 
puffy hands with shiny, taught skin that are not tender on palpation
thickened skin
reduced oral aperture
sclerodactyly (shortening of appendages)
vitiligo
52
Q

3 primary pathophysiological components of scleroderma

A
  1. autoantibodies
  2. obliterative vasculopathy
  3. fibrosis
53
Q

main cytokine driving process of scleroderma

A

TGF-B

54
Q

most common manifestations of scleroderma

A

96% get raynauds

94% are ANA positive

55
Q

compare the disease timelines of diffuse cutaneous and limited cutaneous scleroderma

A

diffuse has a rapidly progressing skin thickness that leads to other manifestations over time, and eventually improves after reaching a peak
limited progresses much more slowly

56
Q

examples of manifestations of scleroderma outside of skin involvement

A 
diffuse:
joint contractures
skeletal myopathy
ILD
myocardial involvement
renal crisis
limited:
digital ischemia
esophageal disease
pulm hypertension
malabsorption
57
Q

what is anti-centromere-Ab (ACA)

A

strongly associated with limited cutaneous systemic sclerosis (seen in 50% of cases)

58
Q

what is CREST syndrome

A 
name CREST has been replaced by lcSSc to emphasize the occurrence of systemic manifestations like pulmonary hypertension
C-calcinosis
R-raynauds
E-esophageal dysmotility
S- sclerodactyly
T- telangectasia
59
Q

associations with ACA

A

female predominance
increased risk for progressive Raynauds and digital ischemia
increased risk of isolated PAH

60
Q

associations with Anti-topoisomerase-1 Ab (Scl-70-Ab)

A

more likely to have dcSSc
less likely to have isolated pulmonary hypertension
patients with early diffuse SSc and anti-Scl70-Ab have high risk of severe ILD (23%) and lower risk of renal crisis (10%)

61
Q

associations with anti-RNA polymerase III-Ab (RNA-pol3)

A

present in 3.4-23% of patients with SSc
rapidly progressive skin thickening which can PREDATE onset of Raynaud’s
sclerodermal renal disease develops in 24-33% of patients (5x all other SSc patients)
only 7% develop significant ILD
strongly associated with cancer

62
Q

how to monitor for sclerodermal renal disease

A

vigilant ambulatory BP monitoring

MM Test 2 (23 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions