CTD part III Flashcards

1
Q

scleroderma GI implications

A

esophageal obsturction
bacterial overgrowth
intestinal pseudoobstruction

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2
Q

most common problem in scleroderma GI

A

hearburn, regurg and dysphagia

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3
Q

things that causes dysphagia for solids and liquids

A

scleroderma
achalasia
diffuse esophgeal spasm

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4
Q

causes of dysphagia for solids only

A

carcinoma
ring- webs
eosiophilic esophagitis
peptic stricture

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5
Q

What is Limited Scleroderma

A

CREST syndrome

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6
Q

higher incidence of what in limited scleroderma

A

pulmonary HTN
anti centromere Ab
many have PBC

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7
Q

What is Shulmans syndrome

A

eosinophilic fascitis

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8
Q

patchy scleroderma like reaction after vigorous exercise

A

eosinophilic fascitis

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9
Q

what else can cause eosinophilic fascitis

A

statins and borrelia

TGF beta and fibrosis

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10
Q

Scleredema is assoc with what

A

DM

sometimes after URI

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11
Q

nephrogenic fibrosing dermopathy affects what

A

skin, muscles, heart and kidneys

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12
Q

how do Dx nephfrogenic fibrosing dermopathy

A

gadolinum– MRI

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13
Q

groove sign

A

eosinophilic fascitis inflammatoin and fibrosis in subcut

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14
Q

what can cause plantar fascitis and polyarthritis syndrome

A

paraneoplastic: adenocarcinomas and hematologic malignancies

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15
Q

characteristics of plantar fascitis and polyarthritis syndrome

A
fibroblas proliferation from tgf beta
pain stiff ness DIP and PIP
thickening of palmar fascia!!!
finger flexion contractures!!
ANA negative
no RP
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16
Q

What can cause eosinophilia myalgia

A

L tryptophan

fever, eos, neurologic manifestations, pulm HTN

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17
Q

what cauese toxic oil syndrome

A

contaminated rapeseed oil

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18
Q

what cause acroosteolysis and vascular lesions and sclerodactylyl

A

vinyl chloride disease

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19
Q

What is polymyositis

A

painless proximal mm weakness

standing up, stiars

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20
Q

what is dermatomyositis

A

painless proximal muscle weakness with preceding rash

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21
Q

what is inclusion body myositis

A

asymmetric distal weakness, thigh atrophy, finger flexor weakness, dysphagia and older men

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22
Q

elevated CPK

A

muscle involvement

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23
Q

dermato and polymyostitis characterizations

A

shoulder hip weakness
elevated enzymes
positive EMG
myofiber degeneration with mononuclear infiltration

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24
Q

what are skin cahgnes in dermatomyositis

A

shawl sign, Gottrons papules, heliotrope eyes, nailfold capillary loops

25
Q

what can cause DM PM environmentally

A

RNA virus with myositis in HLA DRB1 individual

26
Q

dermatomyositis pathophys

A

autoimmune
C’ activation with MAC and lysis endomysial capillaries, capillary necrosis, perivascula inflammation, muscle ischemia
CD4 cells, infiltrating B cells and upregulation MHC I

27
Q

What immune cell is responsible for damage in polymyositis and inclusion body myositis

A

CD8 T cells with perforin and macrophages

perifascicular

28
Q

Shawl sign

A

think malignancy wtih dermatomyositis

29
Q

heliotrope assoc with

A

ovarian breast GI and lung cancer

can be drug induced

30
Q

mechanic hand sign is asso with

A

anti -ARS

31
Q

gottrons papules

A

dermatomyositis

32
Q

shawl sign in women should be screened for

A

adenocarcinomas

33
Q

Ab to anti synthestase anti Jo 1 indicates what organ involvement

A

lung- pulm fibrosis

34
Q

aminoacyl tRNA synthetase

A

severe involvement mm and joints an ILD

35
Q

dypnea and cough in dermatomyositis

A

cardiac and pulmonary

36
Q

CV complications dermatomyositis

A

AV block, MI CHF and diastolic dysfunction

37
Q

how do monitor CV in dermatomyositis

A

MRI and nailfold capillaroscopy vasculopathy

38
Q

are there vegetations in dermatomyositis

A

no

39
Q

GI in dermatomyositis

A

oropharyngeal dysphagia
GERD
vasculitis with GI ulcers

40
Q

ANA useful to distinguish poly and dermato

A

no

41
Q

what mm enzymes elevated in poly and dermato

A

CPK and aldolase

42
Q

How can poly and dermatomyositis mimic hepattiis

A

inc LDH AST ALT

43
Q

What is ASS

A
anti jo anti synthetase syndrome
fever
polyarthritis
RP
mechanics hands
DM rash
pulmonary fibrosis
44
Q

What Ab is associated with worse interstitial lung disease

A

Ro52

45
Q

muscle biopsy of poly or dermatomyositis

A

edema in striated muscle

46
Q

ARS

A

anti aminoacyl tRNA synthetase Ab

47
Q

Anti p155/140 !!!!!

A

cancer associated dermatomyositis
tRNA synthetase Ab
“amyopathic dermatomyositis”

48
Q

what Ab assoc with heliotrope rash and gottrons rash

A

anti SAE

49
Q

anti Mi2

A

good prognosis

50
Q

anti Scl 90

A

severe systemic sclerosis

51
Q

specked anti RNA polymerase2

A

systemic sclerosis

52
Q

B23

A

pulmonary HTN with sclerosis

53
Q

what is MCTD

A

mixed CT disease

54
Q

strong features MCTD

A
RP
no renal or CNS
more severe arthritis +RF and non fibrosis pulm HTN HLADRB1
Anti U1 RNP
anti hnRNP A2
55
Q

Anti U1 RNP

A

combination of any SLE, SS, PSS, DM/PM

56
Q

demographics MCTD

A

women teens or 20s

fever severe myalgia, myocarditis, ILD, dysphagia

57
Q

can;t take patient off ventilator
flaccid paralysis
dec pain and temp

A

critical illness myopathy and polyneuropathy

58
Q

oral Sc sjogrens

A

dental caries

59
Q

immune cell in SS

A

CD4