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

B6.023 Dermatomysositis and Scleroderma Flashcards

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1
Q

what is interface dermatitis?

A

inflammation at the dermal-epidermal junction

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2
Q

histo characteristics of interface dermatitis

A

vacuolar change

lymphocytic inflammation that obscures the D/E junction

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3
Q

differential diagnosis associated with interface dermatitis

A 
SLE
DM 
cutaneous lupus
erythema multiforme
drug eruption
GVHD
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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

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5
Q

skin findings associated with DM

A 
gottrons papules
periungal erythema
nailfold capillary changes
heliotrope rash
V sign
Shawl sign
muscle wasting
calcincosis
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6
Q

gottron’s papules

A

interface dermatitis over IP joints

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7
Q

nailfold capillary changes

A

palisading array of vessels

cause erythematous appearance

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8
Q

V-sign and Shawl sign

A

rash in pattern of photo-distribution

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9
Q

calcinosis

A

nodular or linear densities appearing under the skin

show up on xray

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10
Q

epidemiology of DM

A

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

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11
Q

juvenile DM

A

prominent vasculopathy including GI vasculitis leading to GI bleed or perforation

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12
Q

DM features

A

proximal muscle and skin

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13
Q

polymyositis features

A

absent skin involvement

Jo-1-Ab

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14
Q

inclusion body myositis features

A

older adults
distal extremities involved
poor response to treatment

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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)

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16
Q

pentad of symptoms of antisynthetase syndrome

A 
myositis
ILD**
Raynaud's
inflammatory arthropathy
mechanic's hand
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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

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18
Q

histo of PM

A

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

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19
Q

histo of IBM

A

same as PM +

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

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20
Q

pre treatment assessment of DM

A 
overlapping immune conditions (ILD, SSc, SLE)
comorbid conditions (DM2, liver disease, cancer)
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21
Q

mainstay of DM treatment

A

prednisone
high dose, tapered over months
+ second immunosuppressive agent

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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)

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23
Q

what % of PM and DM are associated with malignancy

A

10%

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

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24
Q

common cancers associated with PM and DM

A

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

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25
antibodies with a positive association with malignancy
antibodies to TIF-1gamma (anti-p155, anti-p155/140) | antibodies to NPX-2 (anti-MJ or anti-p140)
26
antibodies with a negative association with malignancy
anti-synthetase antibodies anti-Mi-2 anti-SRP myositis associated antibodies (anti-RNP, anti-PM-Scl, anti-Ku)
27
overlap CTD
multiple identifiable CTDs
28
mixed CTD
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
undifferentiated CTD
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
what is Raynaud's
an exaggerated response of the digital arterial circulation triggered by cold temperature and emotional stress exaggeration of a normal response
31
epidemiology of Raynauds
present in 3-15% of the normal population more common in women (3-4:1) often beings before age 20
32
discuss the pathophysiology of Raynauds
vasoconstriction at the level of the digital arteries, precapillary arterioles, and/or cutaneous AV shunts
33
thermoregulation of the skin
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
primary Raynauds
very low risk to progress to systemic sclerosis (scleroderma) or another CTD (<1%)
35
therapy for primary Raynauds
focused on conservative measures and dihydropyridine Ca channel blockers when necessary -amlodipine, nifedipine
36
characteristics of primary Raynauds
- 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
characteristics of secondary Raynauds
- 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
asymmetric Raynaud events or single digit only
digital ischemia with vasospasms mimicking RP
39
metabolic disease that could cause Raynauds
hypothyroid
40
progression of nailfold capillary changes in scleroderma
palisading levels of vessels (normal) micro hemorrhages loss of capillary dilated loops disorganization
41
what is scleroderma
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
2 types of scleroderma classifications
localized -skin and soft tissue involvement, no internal organ disease systemic - have systemic manifestations
43
classes of localized scleroderma
morphea linear scleroderma scleroderma en coup de sabre
44
what is a morphea plaque
large plaque presenting with scaling, induration, and redness on the border can appear alone or all over body
45
epidemiology of localized scleroderma
kids > adults
46
results of localized scleroderma
long term morbidity from skin, muscle, and bone atrophy causing growth defects and deformities
47
what is a coup de sabre manifestation
sclerotic line of scar tissue down forehead | can get cranial nerve palsies
48
classes of systemic scleroderma
``` limited cutaneous (lcSSc) diffuse cutaneous (dcSSc) sine scleroderma (no skin involvement) overlap ```
49
distribution of limited cutaneous systemic scleroderma
distal involvement only
50
distribution of diffuse cutaneous systemic scleroderma
involvement anywhere on body
51
manifestations of systemic sclerosis
``` puffy hands with shiny, taught skin that are not tender on palpation thickened skin reduced oral aperture sclerodactyly (shortening of appendages) vitiligo ```
52
3 primary pathophysiological components of scleroderma
1. autoantibodies 2. obliterative vasculopathy 2. fibrosis
53
main cytokine driving process of scleroderma
TGF-B
54
most common manifestations of scleroderma
96% get raynauds | 94% are ANA positive
55
compare the disease timelines of diffuse cutaneous and limited cutaneous scleroderma
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
examples of manifestations of scleroderma outside of skin involvement
``` diffuse: joint contractures skeletal myopathy ILD myocardial involvement renal crisis limited: digital ischemia esophageal disease pulm hypertension malabsorption ```
57
what is anti-centromere-Ab (ACA)
strongly associated with limited cutaneous systemic sclerosis (seen in 50% of cases)
58
what is CREST syndrome
``` 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
associations with ACA
female predominance increased risk for progressive Raynauds and digital ischemia increased risk of isolated PAH
60
associations with Anti-topoisomerase-1 Ab (Scl-70-Ab)
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
associations with anti-RNA polymerase III-Ab (RNA-pol3)
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
how to monitor for sclerodermal renal disease
vigilant ambulatory BP monitoring

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