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MSK II > CT disorders > Flashcards

CT disorders Flashcards

1
Q

Class I autoimmune diseases

A

HLA-A, B, or C

more common in men

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2
Q

Class II autoimmune disease

A

HLA-D

more common in women

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3
Q

SLE

A

inflammatory autoimmune disease characterized by ANA
HLA-B8
HLA-DR2
HLA-DR3
more common in women and non-whites, especially blacks
C’ deficiencies

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4
Q

splinter hemorrhages

A

in fingernails
rare, but can occur in SLE
most likely dt vascular changes in bed of nail

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5
Q

SLE Dx

A

must have 4/11 criteria

B3O1R1N1 with D3ermA1titiS1

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6
Q

B3

A
  • Hematologic disorder (hemolytic anemia, leukopenia, thrombocytopenia)
  • Immunologic Disorder (ds-DNA, Sm, or APA, or FPSTS- false + for syphillis)
  • ANA (def going to be pos- lupus band test)
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7
Q

ANA subtypes

A 
Ro/SSA, La/SSB
Sm (smith)
RNP/U1-RNP
Scl70/topizomerase 1
Jo-1
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8
Q

which pattern is most specific to SLE

A

peripheral/rim

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9
Q

O1R1N1

A
  • oral ulcers (can also be in vagina, nose)
  • renal disorder (proteinuria or casts)
  • neurological disorder (seizures, psychosis)
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10
Q

proteinuria in SLE

A

> 500 or 3+

any type of casts

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11
Q

D3

A

malar rash
discoid rash (DLE)
photosensitivity

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12
Q

A1titiS1

A

arthritis- w/or w/o synovitis

serositis- pleuritis or pericarditis

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13
Q

other cardiac issues of SLE

A
  • alveolar hemorrhage-> cough up blood, only 2 diseases that will cause hematuria and hemoptosis: SLE and goodpastures
  • endocarditis that is not infectious
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14
Q

HCQ

A

reverses platelet activation via IgG antiphospholipid

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15
Q

libman-sacks vegetations aka

A

atypical verrucous vegitations

marantic or non-bacterial thrombotic endocarditis

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16
Q

what drugs are implicated in drug induced SLE

A 
hydralzine
isoniazide
procainamide
methimaxole
PTU
etanercept
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17
Q

drug induced SLE

A 
seen in slow acetylators
no renal disease
no CNS disease
\+anti-histone Abs -> homogenous/diffuse pattern
TQ
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18
Q

which Ab is most dangerous in prego

A

lupus anticoagulant anticardiolipin Ab

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19
Q

what other Abs should you screen for with SLE and prego

A

beta 2 glycoprotein 1
anti-Ro
anti-La

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20
Q

which SLE drug is safe in prego

A

HCQ

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21
Q

how do you differentiate SLE from preeclampsia?

A

both have: HTN, proteinuria, and low platelets
Abnormal LFTS: rare in SLE, common in preeclampsia
serology: dsDNA and low C3/C4 in SLE, nothing in preeclampsia
uric acid: notmal in SLE, high in preeclampsia

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22
Q

describe rash of SLE

A

spares knuckles and face
non-indurated, erythematous plaques to papulosquamous or annular lesions with central hypopigmentation or telaniectasia
seen in sun exposed areas
d/t Ro/SSA

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23
Q

what is the fetus at risk for

A

heart block

Tx with HCQ

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24
Q

which defect is best answer when pt has clotting issues

A

lupus anticoagulant

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25
Q

Antiphospholipid Ab syndrome d/t

A

CTDs (SLE)
malignancy
infections (HIV)
drugs (phenytoin, chlorpromazine)

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26
Q

Dx antiphospholipid Ab syndrome

A

1 clinical + 1 lab for 3 months

clinical: spontaneous aborrtion, vascular thrombosis
lab: anticardiolipin Ab, lupus anticoagulant, anti beta2 glycoprotein 1

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27
Q

how do you confrim antiphospholipid Ab syndrome

A

russel viper venom test

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28
Q

diseases that cause aa and vv clots

A 
TTP
HIT
DIC
ACLA (anticardiolipin Ab)
LA (lupus anticoagulant)
PNH
infective endocarditis
vasculitis
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29
Q

livedo reticularis

A

rash caused by spasm of deeper arterioles

30
Q

livedo reticularis causes

A

cryoglobulins
antiphopholipid syndrome
cholesterol emboli

31
Q

cryoglobulins caused by

A

hep C

sjrogens

32
Q

rash of scleroderma

A

morphea

33
Q

scleroderma aka

A

progressive systemic sclerosis

34
Q

scleroderma symptoms

A
  • Raynauds
  • cutaneous, subQ calcifications, pigmentation/depigmentation, telangiectasias
  • cardiac
  • pulmonary
  • GI
  • renal
35
Q

scleroderma and heart

A

pericarditis
fibrosis
heart block
R-CHF

36
Q

scleroderma and lungs

A

restrictive lung disease with decreased CO diffusion

pulmonary HTN

37
Q

scleroderma and GI

A

GERD
trouble swallowing below sternum
hypomotility w/bacterial overgrowth
large mouth diverticulum

38
Q

scleroderma and renal

A

onion skinning of aa

HTN

39
Q

pathophysiology of scleroderma

A
  • fibroblast metabolic defect with increased collagen
  • vascular injury of endothelium with obliteration (onion skinning)
  • autoimmunity (HLA-DPB1, HLA-DPB2)
  • Silica, solvents
  • paraneoplastic
40
Q

scleroderma Dx

A

1 major or 2/3 minor

can Dx highly likely scleroderma w/3/5 CREST

41
Q

scleroderma major criteria

A

symmetric thickening, tightening, and induration of skin proximal to MTP joints, arms, trunk, face, etc…

42
Q

scleroderma minor criteria

A

sclerodactyly
digital pitting
bibasilar pulmonary fibrosis

43
Q

CREST

A 
calcinosis
raynauds
esophageal dysmotility
sclerodactyly 
telangiectatsia
44
Q

skin findings of sclerederma

A 
raynauds
non-pitting edema with erythema and itching
subQ calcifications
finger tip ulceration
telangiectasia
45
Q

digital ulcers

A

associated with anti-topoisomerase

46
Q

finger loss

A

associated with anti-centromere

47
Q

primary raynauds

A

<30
symmetrical
normal SR
no nail bed capillary changes (if capillary changes secondary)

48
Q

secondary capillary changes

A 
CT disease (scleroderma)
drugs/toxins
structural aa disease
occupational
hemorrheologic  
pheochromocytoma
hypothyroidism
49
Q

Tx of raynauds

A 
life style
CaCh blockers
topical nitrates
phosphodiesterase inhibitors
infusion of prostacyclin 
if they have ulcers -> endothelin receptor antagonist
50
Q

scleroderma MSK changes

A

tendon friction rubs
joint stiffness
restricted ROM
Tx with MTX

51
Q

anti-RNA polymerase III

A

compatible w/speckled ANA
marker for renal disease, prone to HTN and onion skinning
can be Tx with ACE inhibitors

52
Q

what antibody is associated with interstital lung disease in scleroderma

A

anti-topoisomerase/anti SCL-70

LEADING COD in scleroderma pts

53
Q

pulmonary HTN and scleroderma

A

seen more in CREST then full scleroderma

risk factors include telangiectasia and anti-centromere Ab and anti-B23

54
Q

scleroderma like syndromes

A

tend to lack raynauds, sclerodactyly, and nail capillary changes

55
Q

types of scleroderma like syndromes

A 
eosinophilic fascitis
scleodema
scleromyxedema
nephrogenic fibrosing dermpathy
plantar fascitis and polyarthritis syndrome
56
Q

eosinophilic fascitis

A 
aka shulmans syndrome 
follows vigorous exercise
associated with statins and borrelia 
spares hands
M proteins
groove sign/ woody texture of limbs
57
Q

sclerodema

A

no esophageal involvement, but may involve tongue
associated with DM
may follow acute URI
M proteins

58
Q

nephrogenic fibrosing dermopathy

A

gadolium from MRIs

no Abs

59
Q

plantar fasciitis and polyarthritis syndrome

A 
paraneoplastic
TGF-beta
thickening of palmar fascia with contractures
NO raynauds
hand pain and stiffness
ANA neg
60
Q

dermatomyositis

A

Need 4/5:

  • shoulder and hip weakness
  • elevated enzymes
  • positive EMG
  • myofiber degeneration w/mononuclear infiltration
  • skin changes
61
Q

polymyositis

A

more severe weakness
no derm changes
cardiac involvement

62
Q

inclusion body myositis

A 
asymmetric distal weakness
thigh atropy
finger flexor weakness
dysphagia
older men
63
Q

dermatomyositis pathogenesis

A

C’ activation -> MAC
CD4 cells
immune attack is on microvasculature

64
Q

polymyositis and inclusion body myositis pathogenesis

A

CD8 cells

in polymyositis immune attach is on myofibers which express HMD1 Ags on saroclemma

65
Q

dermatomyositis and malignancy

A

shawl sign

anti-155/140

66
Q

anti-Jo/anti-synthetase

A 
bad
fever
arthritis
raynauds
mechanics hands
dermatomyositis rash
pulmonary fibrosis
67
Q

symptoms of dermatomyositis

A 
proximal mm weakness (cannot climb stairs/get up from chair)
proximal dysphagia
shawl sign
heliotrope rash
gottrons patches on knuckles
mechanics hands
68
Q

mixed CT disease

A 
raynauds
no renal or CNS disease
more severe arthritis (RF +)
non fibrosis pulmonary HTN
anti-U1-RNP
69
Q

sjogrens pathogenesis

A

CD4 cells attack glands
can spread to kidneys, liver or lungs
can lead to vasculitis -> cyroglobulins

70
Q

Dx sjogrens

A 
4/6:
dry eyes
oral- dry and carries
occular signs (schirmers/rose bengal)
salivary involvement 
characterisitc histo
RF,SS-A, SS-B
71
Q

causes of stridor

A

ventilator trauma
wegners
relapsing polychondritis

72
Q

whipples

A

prodromal migratory arthritis followed by diarrhea and weight loss
PAS+
macros

MSK II (19 decks)

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