CTD part III

Question 1

scleroderma GI implications

Answer 1

esophageal obsturction
bacterial overgrowth
intestinal pseudoobstruction

Question 2

most common problem in scleroderma GI

Answer 2

hearburn, regurg and dysphagia

Question 3

things that causes dysphagia for solids and liquids

Answer 3

scleroderma
achalasia
diffuse esophgeal spasm

Question 4

causes of dysphagia for solids only

Answer 4

carcinoma
ring- webs
eosiophilic esophagitis
peptic stricture

Question 5

What is Limited Scleroderma

Answer 5

CREST syndrome

Question 6

higher incidence of what in limited scleroderma

Answer 6

pulmonary HTN
anti centromere Ab
many have PBC

Question 7

What is Shulmans syndrome

Answer 7

eosinophilic fascitis

Question 8

patchy scleroderma like reaction after vigorous exercise

Answer 8

eosinophilic fascitis

Question 9

what else can cause eosinophilic fascitis

Answer 9

statins and borrelia

TGF beta and fibrosis

Question 10

Scleredema is assoc with what

Answer 10

DM

sometimes after URI

Question 11

nephrogenic fibrosing dermopathy affects what

Answer 11

skin, muscles, heart and kidneys

Question 12

how do Dx nephfrogenic fibrosing dermopathy

Answer 12

gadolinum– MRI

Question 13

groove sign

Answer 13

eosinophilic fascitis inflammatoin and fibrosis in subcut

Question 14

what can cause plantar fascitis and polyarthritis syndrome

Answer 14

paraneoplastic: adenocarcinomas and hematologic malignancies

Question 15

characteristics of plantar fascitis and polyarthritis syndrome

Answer 15

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

Question 16

What can cause eosinophilia myalgia

Answer 16

L tryptophan

fever, eos, neurologic manifestations, pulm HTN

Question 17

what cauese toxic oil syndrome

Answer 17

contaminated rapeseed oil

Question 18

what cause acroosteolysis and vascular lesions and sclerodactylyl

Answer 18

vinyl chloride disease

Question 19

What is polymyositis

Answer 19

painless proximal mm weakness

standing up, stiars

Question 20

what is dermatomyositis

Answer 20

painless proximal muscle weakness with preceding rash

Question 21

what is inclusion body myositis

Answer 21

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

Question 22

elevated CPK

Answer 22

muscle involvement

Question 23

dermato and polymyostitis characterizations

Answer 23

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

Question 24

what are skin cahgnes in dermatomyositis

Answer 24

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

Question 25

what can cause DM PM environmentally

Answer 25

RNA virus with myositis in HLA DRB1 individual

Question 26

dermatomyositis pathophys

Answer 26

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

Question 27

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

Answer 27

CD8 T cells with perforin and macrophages | perifascicular

Question 28

Shawl sign

Answer 28

think malignancy wtih dermatomyositis

Question 29

heliotrope assoc with

Answer 29

ovarian breast GI and lung cancer | can be drug induced

Question 30

mechanic hand sign is asso with

Answer 30

anti -ARS

Question 31

gottrons papules

Answer 31

dermatomyositis

Question 32

shawl sign in women should be screened for

Answer 32

adenocarcinomas

Question 33

Ab to anti synthestase anti Jo 1 indicates what organ involvement

Answer 33

lung- pulm fibrosis

Question 34

aminoacyl tRNA synthetase

Answer 34

severe involvement mm and joints an ILD

Question 35

dypnea and cough in dermatomyositis

Answer 35

cardiac and pulmonary

Question 36

CV complications dermatomyositis

Answer 36

AV block, MI CHF and diastolic dysfunction

Question 37

how do monitor CV in dermatomyositis

Answer 37

MRI and nailfold capillaroscopy vasculopathy

Question 38

are there vegetations in dermatomyositis

Answer 38

no

Question 39

GI in dermatomyositis

Answer 39

oropharyngeal dysphagia GERD vasculitis with GI ulcers

Question 40

ANA useful to distinguish poly and dermato

Answer 40

no

Question 41

what mm enzymes elevated in poly and dermato

Answer 41

CPK and aldolase

Question 42

How can poly and dermatomyositis mimic hepattiis

Answer 42

inc LDH AST ALT

Question 43

What is ASS

Answer 43

``` anti jo anti synthetase syndrome fever polyarthritis RP mechanics hands DM rash pulmonary fibrosis ```

Question 44

What Ab is associated with worse interstitial lung disease

Answer 44

Ro52

Question 45

muscle biopsy of poly or dermatomyositis

Answer 45

edema in striated muscle

Question 46

ARS

Answer 46

anti aminoacyl tRNA synthetase Ab

Question 47

Anti p155/140 !!!!!

Answer 47

cancer associated dermatomyositis tRNA synthetase Ab "amyopathic dermatomyositis"

Question 48

what Ab assoc with heliotrope rash and gottrons rash

Answer 48

anti SAE

Question 49

anti Mi2

Answer 49

good prognosis

Question 50

anti Scl 90

Answer 50

severe systemic sclerosis

Question 51

specked anti RNA polymerase2

Answer 51

systemic sclerosis

Question 52

B23

Answer 52

pulmonary HTN with sclerosis

Question 53

what is MCTD

Answer 53

mixed CT disease

Question 54

strong features MCTD

Answer 54

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

Question 55

Anti U1 RNP

Answer 55

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

Question 56

demographics MCTD

Answer 56

women teens or 20s | fever severe myalgia, myocarditis, ILD, dysphagia

Question 57

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

Answer 57

critical illness myopathy and polyneuropathy

Question 58

oral Sc sjogrens

Answer 58

dental caries

Question 59

immune cell in SS

Answer 59

CD4