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MSK Exam 2 Spring 2015 > Connective Tissue Disease - Darrow > Flashcards

Connective Tissue Disease - Darrow Flashcards

1
Q

autoimmune diseases

A

linked to HLA antigens

class I - A, B, C
class II - D
class I - men
class II - female
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2
Q

SLE

A

in black women
ANA, HLA-B8, DR2 and DR3 positive

seen with complement deficiency

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3
Q

arthralgia, rash on face, lung field rub, proteinuria, anemia

A

SLE

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4
Q

elevated ESR and normal CRP

A

in SLE

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5
Q

pericarditis

A

in SLE

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6
Q

diagnosis of SLE

A

requires 4 of 11 criteria

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7
Q

criteria for diagnosis of SLE

A

B3 O1 R1 N1
with
D3ermA1titiS1

B3 - blood
1 - heme disorder - anemia, leukopenia, thrombocytopenia
2 - immunologic disorder - ds-DNA, Sm, APA, FPSTS
3 - ANA

O1
4 - oral ulcers

R1
5 - renal disorders - proteinuria or cellular casts

N1
6 - neurologic disorders - seizure or psychosis

D3
7 - malar rash
8 - discoid lupus
9 - photosensitivity

A1
10 - arthritis

S1
11 - serositis - pleuritis or pericarditis

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8
Q

speckled and rim pattern

A

in SLE

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9
Q

seizure of psychosis

A

in SLE

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10
Q

smoking

A

makes SLE rash worse

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11
Q

cough up blood

A

alveolar hemorrhage

-associated with SLE

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12
Q

non-infectious endocarditis

A

libman sacks

-associated with SLE

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13
Q

tx of SLE

A

hydroxychloroquine

  • fight SLE
  • stop anti-phospholipid Ab formation
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14
Q

before making diagnosis of SLE in patient

A

exclude drug induced lupus

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15
Q

splinter hemorrhages

A

with bacterial endocarditis

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16
Q

drug induced lupus

A

slow acetylators - if inhibit DNA methylation T cells - become autoreactive

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17
Q

sx with drug induced lupus

A

no renal disease
no CNS disease

+ anti-histone Abs

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18
Q

drugs triggering autoimmunogenicity

A 
hydralazine
isoniazid
procainamide
methimazole
prophylthiouracil
etanercept
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19
Q

anti-histone Abs

A

drug induced lupus

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20
Q

CI in pregnancy

A

CI in pregnancy

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21
Q

safe in pregnancy

A

azathioprine and HCQ

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22
Q

SLE vs. preeclampsia

A

abnormal liver test and high uric acid - preeclampsia

dsDNA, low C3/C4 and normal uric acid and liver tests - SLE

both have HTN, proteinuria, and thrombocytopenia

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23
Q

RO/SSA patient daughter born with

A

heart block

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24
Q

ring like or snake like plaque with central clearing

A

SCSLE - subacute cutaneous lupus erythematosis
-characteristic rash

RO-SSA and heart block in offspring

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25
Q

lupus anticoagulant

A

worst prognosis

venous and arterial clotting

antiphospholipid antibody syndrome

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26
Q

diagnosis of antiphospholipid antibody syndrome

A

1 clinical and 1 lab criteria - 3 months apart

lab

  • anticardiolipid Ab
  • lupus anticoagulant
  • anti-beta2 glycoprotein 1

clinical

  • spontaneous abortion
  • vascular abortion
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27
Q

best correlation with thrombotic events in antiphospholipid Ab syndorme

A

lupus anticoagulant

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28
Q

russel viper venom test

A

confirmation of antiphospholipid Ab syndrome

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29
Q

tx with hydroxychloroquine

A

need to do retinal screening

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30
Q

small vessel vasculitis

A

cryoglobulinemia

giraffe type rash - livedo reticularis

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31
Q

cause of livedo reticularis

A

cryoglobulinemia

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32
Q

tightening skin and fibrosis

A

scleroderma

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33
Q

progressive systemic sclerosis

A

scleroderma

-systemic

34
Q

onion skinning of renal arteries

A

scleroderma

35
Q

GI sx of scleroderma

A

stay below sternum

36
Q

fibroblast metabolic defect with increased collagen

A

scleroderma

autoimmunity

can be caused by silica**

37
Q

diagnosis of scleroderma

A

1 major or 2 of 3 minor

major - thick, tight, induration of skin proximal to MCP

minor

  • sclerodactyly
  • digital pitting
  • bibasilar pulmonary fibrosis
38
Q

high probability of scleroderma

A

with 3 of 5 crest featuresq

39
Q

first sign of scleroderma

A

raynauds

40
Q

signs of scleroderma

A 
raynauds
nonpitting edema
subQ calcifications
finger tip ulcerations
telangiectasia
41
Q

tendon friction rub

A

scleroderma

42
Q

raynauds

A

female

-precipitated with cold, stress, drugs

43
Q

finger loss

A

anti-centromere

44
Q

digital ulcer

A

anti-topoisomerase

45
Q

<30yo, normal sed rate, no nail bed capillary changes

A

primary raynauds

46
Q

visceral changes in scleroderma

A

linked to extent of skin thickening

47
Q

what kills you in scleroderma

A

kidney failure with HTN
-until ACE inhibitors

now - lung issues kill you

48
Q

anti-RNA polymerase III

A

bad Ab in scleroderma

onion skinning - renal disease**

49
Q

stop progression of scleroderma

A

ACE inhibitors

50
Q

anit-SCL-70

A

scleroderma interstitial lung disease**

51
Q

scleroderma risk fx

A

AA

52
Q

anti-centromere Ab and anti-B23

A

scleroderma pulmonary HTN

53
Q

dysphagia cause

A

scleroderma

for solids and liquids

54
Q

limited scleroderma

A

CREST syndrome

55
Q

induces fibrosing dermopathy

A

gadolinum

prep for X-ray or CAT scan

56
Q

trauma, following exercise, M protein

A

eosinophilic fasciitis

57
Q

shulmans syndrome

A

eosinophilic fasciitis

58
Q

plantar fascitia and polyarthritis syndrome

A

associated with malignancy**

is a paraneoplastic syndrome

fibroblast proliferation due to TGF-B

pain, stiff, swollen DIP and PIP
thick palmar fascia
thick plantar fascia
ANA negative
no RP
59
Q

polymyositis and dermatomyositis

A

proximal muscle weakness

60
Q

inclusion body myositis

A

distal muscle weakness

61
Q

dermatomyositis

A

CD4 - B cells

62
Q

polymyositis and inclusion body myositis

A

CD8 T cells

63
Q

very high CPK

A

dermatomyositis

64
Q

heliotrope rash, shawl sign, gottrons papules, nailfold capillary loop

A

dermatomyositis

65
Q

scleroderma dysphagia

A

low in esophagus - smooth m

66
Q

dermatomyositis dysphagia

A

high in esophagus - skeletal m

67
Q

specific diagnosis of dermatomyositis

A

perivascular infiltrate with cap necrosis and fiber destruction

68
Q

shawl sign

A

in dermatomyositis

association with malignancy**

69
Q

anti-Jo1 dermatomyositis

A

lung involvement**

70
Q

anti-p155/140

A

associated with cancer

also shawl sign

71
Q

anti-U1 RNP

A

mixed connective tissue disease

-aka overlap syndrome

72
Q

pt on ventilator, sepsis forms, high blucose, flaccid distal and proximal muscle groups, absent DTRs

A

critical illness myopathy neuropathy

73
Q

sjogrens

A

T cell CD4 - infiltrate gland

anti-Ro and anti-La produced by B cells

74
Q

cryoglobulins

A

small vessel vasculitis in sjogrens

75
Q

Ab to type II collagen

A

relapsing polychondritis

polyarthritis across wrist and MCPs, also eye and ear involvement

76
Q

IgG4 releated disease

A

relapsing polychondritis

77
Q

obliterative phlebitis

A

inflammation of well of vein that lumen obstructed

in relapsing polychondritis

IgG4 related disease

78
Q

large joint arthritis, night sweats, anorexia, diarrhea, weight loss, lymphadenopathy, hepatosplenomegaly, hyperpigmentation

A

whipples disease

79
Q

stages of whipples

A

prodrome - migratory arthritis

steady state - diarrhea and weight loss

80
Q

PAS positive macrophages

A

whipples disease

81
Q

hyperpigmentation

A

niacin decrease

aka pellagra

MSK Exam 2 Spring 2015 (10 decks)

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