CTD part III Flashcards
scleroderma GI implications
esophageal obsturction
bacterial overgrowth
intestinal pseudoobstruction
most common problem in scleroderma GI
hearburn, regurg and dysphagia
things that causes dysphagia for solids and liquids
scleroderma
achalasia
diffuse esophgeal spasm
causes of dysphagia for solids only
carcinoma
ring- webs
eosiophilic esophagitis
peptic stricture
What is Limited Scleroderma
CREST syndrome
higher incidence of what in limited scleroderma
pulmonary HTN
anti centromere Ab
many have PBC
What is Shulmans syndrome
eosinophilic fascitis
patchy scleroderma like reaction after vigorous exercise
eosinophilic fascitis
what else can cause eosinophilic fascitis
statins and borrelia
TGF beta and fibrosis
Scleredema is assoc with what
DM
sometimes after URI
nephrogenic fibrosing dermopathy affects what
skin, muscles, heart and kidneys
how do Dx nephfrogenic fibrosing dermopathy
gadolinum– MRI
groove sign
eosinophilic fascitis inflammatoin and fibrosis in subcut
what can cause plantar fascitis and polyarthritis syndrome
paraneoplastic: adenocarcinomas and hematologic malignancies
characteristics of plantar fascitis and polyarthritis syndrome
fibroblas proliferation from tgf beta pain stiff ness DIP and PIP thickening of palmar fascia!!! finger flexion contractures!! ANA negative no RP
What can cause eosinophilia myalgia
L tryptophan
fever, eos, neurologic manifestations, pulm HTN
what cauese toxic oil syndrome
contaminated rapeseed oil
what cause acroosteolysis and vascular lesions and sclerodactylyl
vinyl chloride disease
What is polymyositis
painless proximal mm weakness
standing up, stiars
what is dermatomyositis
painless proximal muscle weakness with preceding rash
what is inclusion body myositis
asymmetric distal weakness, thigh atrophy, finger flexor weakness, dysphagia and older men
elevated CPK
muscle involvement
dermato and polymyostitis characterizations
shoulder hip weakness
elevated enzymes
positive EMG
myofiber degeneration with mononuclear infiltration
what are skin cahgnes in dermatomyositis
shawl sign, Gottrons papules, heliotrope eyes, nailfold capillary loops
what can cause DM PM environmentally
RNA virus with myositis in HLA DRB1 individual
dermatomyositis pathophys
autoimmune
C’ activation with MAC and lysis endomysial capillaries, capillary necrosis, perivascula inflammation, muscle ischemia
CD4 cells, infiltrating B cells and upregulation MHC I
What immune cell is responsible for damage in polymyositis and inclusion body myositis
CD8 T cells with perforin and macrophages
perifascicular
Shawl sign
think malignancy wtih dermatomyositis
heliotrope assoc with
ovarian breast GI and lung cancer
can be drug induced
mechanic hand sign is asso with
anti -ARS
gottrons papules
dermatomyositis
shawl sign in women should be screened for
adenocarcinomas
Ab to anti synthestase anti Jo 1 indicates what organ involvement
lung- pulm fibrosis
aminoacyl tRNA synthetase
severe involvement mm and joints an ILD
dypnea and cough in dermatomyositis
cardiac and pulmonary
CV complications dermatomyositis
AV block, MI CHF and diastolic dysfunction
how do monitor CV in dermatomyositis
MRI and nailfold capillaroscopy vasculopathy
are there vegetations in dermatomyositis
no
GI in dermatomyositis
oropharyngeal dysphagia
GERD
vasculitis with GI ulcers
ANA useful to distinguish poly and dermato
no
what mm enzymes elevated in poly and dermato
CPK and aldolase
How can poly and dermatomyositis mimic hepattiis
inc LDH AST ALT
What is ASS
anti jo anti synthetase syndrome fever polyarthritis RP mechanics hands DM rash pulmonary fibrosis
What Ab is associated with worse interstitial lung disease
Ro52
muscle biopsy of poly or dermatomyositis
edema in striated muscle
ARS
anti aminoacyl tRNA synthetase Ab
Anti p155/140 !!!!!
cancer associated dermatomyositis
tRNA synthetase Ab
“amyopathic dermatomyositis”
what Ab assoc with heliotrope rash and gottrons rash
anti SAE
anti Mi2
good prognosis
anti Scl 90
severe systemic sclerosis
specked anti RNA polymerase2
systemic sclerosis
B23
pulmonary HTN with sclerosis
what is MCTD
mixed CT disease
strong features MCTD
RP no renal or CNS more severe arthritis +RF and non fibrosis pulm HTN HLADRB1 Anti U1 RNP anti hnRNP A2
Anti U1 RNP
combination of any SLE, SS, PSS, DM/PM
demographics MCTD
women teens or 20s
fever severe myalgia, myocarditis, ILD, dysphagia
can;t take patient off ventilator
flaccid paralysis
dec pain and temp
critical illness myopathy and polyneuropathy
oral Sc sjogrens
dental caries
immune cell in SS
CD4
