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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
Cā€™ 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
Antiphospholipid Ab syndrome d/t
CTDs (SLE) malignancy infections (HIV) drugs (phenytoin, chlorpromazine)
26
Dx antiphospholipid Ab syndrome
1 clinical + 1 lab for 3 months clinical: spontaneous aborrtion, vascular thrombosis lab: anticardiolipin Ab, lupus anticoagulant, anti beta2 glycoprotein 1
27
how do you confrim antiphospholipid Ab syndrome
russel viper venom test
28
diseases that cause aa and vv clots
``` TTP HIT DIC ACLA (anticardiolipin Ab) LA (lupus anticoagulant) PNH infective endocarditis vasculitis ```
29
livedo reticularis
rash caused by spasm of deeper arterioles
30
livedo reticularis causes
cryoglobulins antiphopholipid syndrome cholesterol emboli
31
cryoglobulins caused by
hep C | sjrogens
32
rash of scleroderma
morphea
33
scleroderma aka
progressive systemic sclerosis
34
scleroderma symptoms
- Raynauds - cutaneous, subQ calcifications, pigmentation/depigmentation, telangiectasias - cardiac - pulmonary - GI - renal
35
scleroderma and heart
pericarditis fibrosis heart block R-CHF
36
scleroderma and lungs
restrictive lung disease with decreased CO diffusion | pulmonary HTN
37
scleroderma and GI
GERD trouble swallowing below sternum hypomotility w/bacterial overgrowth large mouth diverticulum
38
scleroderma and renal
onion skinning of aa | HTN
39
pathophysiology of scleroderma
- fibroblast metabolic defect with increased collagen - vascular injury of endothelium with obliteration (onion skinning) - autoimmunity (HLA-DPB1, HLA-DPB2) - Silica, solvents - paraneoplastic
40
scleroderma Dx
1 major or 2/3 minor | can Dx highly likely scleroderma w/3/5 CREST
41
scleroderma major criteria
symmetric thickening, tightening, and induration of skin proximal to MTP joints, arms, trunk, face, etc...
42
scleroderma minor criteria
sclerodactyly digital pitting bibasilar pulmonary fibrosis
43
CREST
``` calcinosis raynauds esophageal dysmotility sclerodactyly telangiectatsia ```
44
skin findings of sclerederma
``` raynauds non-pitting edema with erythema and itching subQ calcifications finger tip ulceration telangiectasia ```
45
digital ulcers
associated with anti-topoisomerase
46
finger loss
associated with anti-centromere
47
primary raynauds
<30 symmetrical normal SR no nail bed capillary changes (if capillary changes secondary)
48
secondary capillary changes
``` CT disease (scleroderma) drugs/toxins structural aa disease occupational hemorrheologic pheochromocytoma hypothyroidism ```
49
Tx of raynauds
``` life style CaCh blockers topical nitrates phosphodiesterase inhibitors infusion of prostacyclin if they have ulcers -> endothelin receptor antagonist ```
50
scleroderma MSK changes
tendon friction rubs joint stiffness restricted ROM Tx with MTX
51
anti-RNA polymerase III
compatible w/speckled ANA marker for renal disease, prone to HTN and onion skinning can be Tx with ACE inhibitors
52
what antibody is associated with interstital lung disease in scleroderma
anti-topoisomerase/anti SCL-70 | LEADING COD in scleroderma pts
53
pulmonary HTN and scleroderma
seen more in CREST then full scleroderma | risk factors include telangiectasia and anti-centromere Ab and anti-B23
54
scleroderma like syndromes
tend to lack raynauds, sclerodactyly, and nail capillary changes
55
types of scleroderma like syndromes
``` eosinophilic fascitis scleodema scleromyxedema nephrogenic fibrosing dermpathy plantar fascitis and polyarthritis syndrome ```
56
eosinophilic fascitis
``` aka shulmans syndrome follows vigorous exercise associated with statins and borrelia spares hands M proteins groove sign/ woody texture of limbs ```
57
sclerodema
no esophageal involvement, but may involve tongue associated with DM may follow acute URI M proteins
58
nephrogenic fibrosing dermopathy
gadolium from MRIs | no Abs
59
plantar fasciitis and polyarthritis syndrome
``` paraneoplastic TGF-beta thickening of palmar fascia with contractures NO raynauds hand pain and stiffness ANA neg ```
60
dermatomyositis
Need 4/5: - shoulder and hip weakness - elevated enzymes - positive EMG - myofiber degeneration w/mononuclear infiltration - skin changes
61
polymyositis
more severe weakness no derm changes cardiac involvement
62
inclusion body myositis
``` asymmetric distal weakness thigh atropy finger flexor weakness dysphagia older men ```
63
dermatomyositis pathogenesis
C' activation -> MAC CD4 cells immune attack is on microvasculature
64
polymyositis and inclusion body myositis pathogenesis
CD8 cells | in polymyositis immune attach is on myofibers which express HMD1 Ags on saroclemma
65
dermatomyositis and malignancy
shawl sign | anti-155/140
66
anti-Jo/anti-synthetase
``` bad fever arthritis raynauds mechanics hands dermatomyositis rash pulmonary fibrosis ```
67
symptoms of dermatomyositis
``` proximal mm weakness (cannot climb stairs/get up from chair) proximal dysphagia shawl sign heliotrope rash gottrons patches on knuckles mechanics hands ```
68
mixed CT disease
``` raynauds no renal or CNS disease more severe arthritis (RF +) non fibrosis pulmonary HTN anti-U1-RNP ```
69
sjogrens pathogenesis
CD4 cells attack glands can spread to kidneys, liver or lungs can lead to vasculitis -> cyroglobulins
70
Dx sjogrens
``` 4/6: dry eyes oral- dry and carries occular signs (schirmers/rose bengal) salivary involvement characterisitc histo RF,SS-A, SS-B ```
71
causes of stridor
ventilator trauma wegners relapsing polychondritis
72
whipples
prodromal migratory arthritis followed by diarrhea and weight loss PAS+ macros

MSK II (19 decks)

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