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CPR II Exam 1 > Connective Tissue Disorders and Vasculitides > Flashcards

Connective Tissue Disorders and Vasculitides Flashcards

1
Q

IgA Vasculitis is common in what age group?

A

kids

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2
Q

what are the symptoms associated with IgA V?

A

tetrad: 1) palpable purpura (with no thrombocytopenia) 2) arthritis/arthralgia 3)abdominal pain 4) renal disease

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3
Q

how do you diagnose IgAV?

A

biopsy, which will show IgA deposits

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4
Q

how do you treat IgAV?

A

supportive and glucocorticoids

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5
Q

how does a patient with anti-GBM present/ have history of?

A

hematuria or hemoptysis

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6
Q

if anti-GBM is not treated, what is the most likely cause of death?

A

pulmonary hemorrhage

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7
Q

how do you diagnose anti-GBM?

A

biopsy (renal usually), which shows deposition of anti-basement membrane autoantibodies

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8
Q

how do you treat anti-GBM?

A

plasmapheresis (removes abs), glucocorticoids, cyclophosphamide, dialysis sometimes

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9
Q

what are the hallmarks of granulomatosis with polyangiitis?

A

granulomatous inflammation, necrotizing vasculitis, segmental glomerulonephritis, nasal involvement (saddle nose)

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10
Q

how do you diagnose granulomatosis polyangiitis?

A

history/physical, serology: ANCA, biopsy: vessel changes with granulomas

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11
Q

how do you treat granulomatosis polyangiitis?

A

no smoking and high dose glucocorticoids

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12
Q

what are the hallmarks of EGPA?

A

asthma+ eosinophilia–> vasculitis with granulomas

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13
Q

what are the phases of EGPA?

A
  1. prodromal phase 2. eosinophilia-tissue infiltration phase 3. vasculitis phase
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14
Q

how do you diagnosed EGPA?

A

CBC with high eosinophilia count serology: ANCA: pANCA, lung biopsy shows granulomas and vascular changes, eosinophils in tissues

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15
Q

how do you treat EGPA?

A

no smoking and glucocorticoids

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16
Q

where is behcet syndrome more commonly seen?

A

turkey, asia, and middle east

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17
Q

how does behcet syndrome present when it affects the large vessels?

A

aneurysms

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18
Q

how does behcet syndrome present when it affects the veins?

A

DVTs

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19
Q

how does behcet syndrome present when it affects the small vessels?

A

triad: 1. recurrent mouth ulcers 2. genital ulcers 3. eye inflammation

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20
Q

what is pathergy and what disease is it associated with?

A

pustules at the site of sterile needle pricks; behcet syndrome

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21
Q

how do you diagnose behcet syndrome?

A

history and physical exam/ serology: HLA-B51

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22
Q

how do you treat behcet syndrome?

A

low dose glucocorticoids

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23
Q

how do you diagnose buerger disease?

A

angiography “corkscrew appearance”

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24
Q

how do you treat Buerger disease?

A

STOP SMOKING- glucocorticoids and anticoagulation do not work

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25
Q

what is polyarteritis nodosa associated with?

A

HBV

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26
Q

how does polyarteritis nodosa affect the skin?

A

livedo reticularis, subcutaneous nodules, ulcers, and digital gangrene

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27
Q

how does polyarteritis nodosa affect the peripheral nerves?

A

80% have vasculitis neuropathy–> mononeuritis multiplex (foot drop)

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28
Q

how does polyarteritis nodosa affect the lungs?

A

it doesn’t–> they are spared

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29
Q

how do you diagnose polyarteritis nodosa?

A

biopsy (fibrinoid necrosis and no granulomas) or angiogram–> micro-aneurysm

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30
Q

how do you treat polyarteritis nodosa?

A

glucocorticoids and treat HBV

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31
Q

how does kowasaki syndrome present?

A

less than 5 years old M>F; Japan; fever, lymphadenopathy, rash strawberry tongue

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32
Q

if kowasaki syndrome goes untreated, how do you die?

A

from coronary involvement –> aneurysm or MI

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33
Q

how do you treat kowasaki syndrome?

A

IVIG within 10 days of symptoms and high does of ASA

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34
Q

who is at most risk of takayasu arteritis?

A

less than 40 year old females, most common in asians

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35
Q

how can takayasu arteritis be described?

A

pulseless disease- obliterate UE peripheral pulses

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36
Q

how does takayasu affect the body?

A

pulmonary involvement, renal artery stenosis leading to HTN, retinopathy (copper wiring infarctions can be seen), and aortic complications: dilations, regurgitation, aneurysm, rupture

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37
Q

how do you diagnose takayasu arteritis?

A

MRI or CT angiography showing long smooth tapered stenosis; biopsy shows granuloma with some giant cells

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38
Q

how do you treat takayasu arteritis?

A

glucocorticoids

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39
Q

giant cell arteritis frequently coexists with what?

A

polymyalgia rheumatica (PMR)

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40
Q

who is more at risk for getting GCA and PMR?

A

females, 40-50, whites>blacks

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41
Q

what might the clinical history/symptoms be in a person with GCA?

A

headache, jaw claudication, visual abnormalities

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42
Q

how do you diagnose GCA?

A

increased ESR, serology: HLA-DR4, temporal artery biopsy is a gold standard

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43
Q

what does a temporal artery biopsy show in a patient with GCA?

A

segmental granulomatous vasculitis with multinucleated giant cells

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44
Q

how do you treat GCA?

A

start glucocorticoids (before biopsy)

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45
Q

what happens if GCA is not treated?

A

blindeness

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46
Q

how does polymyalgia rheumatica (PMR) present?

A

stiffness, soreness, and muscle pain; feelings of weakness as a result of pain but not the objective weakness

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47
Q

how do you diagnose PMR?

A

everything is normal except ESR and CRP

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48
Q

how do you treat PMR?

A

glucocorticoids

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49
Q

how do you diagnose raynaud’s phenomenon?

A

Nailfold capillaroscopy: will be normal in primary. secondary: distorted with widened and irregular loops, dilated lumen and areas of vascular dropout

50
Q

what type of hypersensitivity is SLE?

A

type III

51
Q

what are the hematologic effects of SLE?

A

anemia, thrombocytopenia, venous or arterial thrombosis

52
Q

what are the cardiopulmonary effects of SLE?

A

pericarditis, pleuritis, myocarditis, libman-sacks endocarditis, pts also at risk of MI due to accelerated atherosclerosis

53
Q

what are the neurological effects of SLE?

A

retinopathy (cotton wool spots)

54
Q

what serology testing is used for diagnosing SLE?

A

dsDNA, smith, and anti-Ro and LA

55
Q

which serology test correlates with disease activity of SLE?

A

dsDNA

56
Q

when might complement levels suggest SLE?

A

if C3 and C4 are decreased

57
Q

how do you treat SLE?

A

NSAIDs, avoid sun exposure, glucocorticoids, hydroxychloroquine

58
Q

what should be considered in the management of SLE?

A

you should minimize other conventional risk factors for atherosclerosis–> avoid or quit smoking

59
Q

in patients with long term corticosteroid use, what should you monitor for?

A

avascular necrosis of bone and osteoporosis (DEXA)

60
Q

what are the complications associated with SLE early on?

A

infections and kidney/CNS disease

61
Q

what are the complications associated with SLE later on?

A

accelerated atherosclerosis and thromboembolic events

62
Q

what are the three primary antibodies involved in antiphospholipid antibody syndrome?

A

anti-cardiolipin antibodies (aCL), lupus anticoagulant (LA) and beta 2 glycoprotein 1 (anti-B2 GP1)

63
Q

what is the treatment for anti-phospholipid antibody syndrome?

A

indefinite systemic anticoagulation

64
Q

what effect does DIL have on the body?

A

it promotes demethylation of DNA

65
Q

how do you diagnose DIL?

A

(+) ANA and (+) anti-histone antibodies

66
Q

who is affected by neonatal lupus?

A

it affects children born of mothers with anti-Ro (SSA) antibodies

67
Q

how does neonatal lupus present?

A

rashes, thrombocytopenia, hemolytic anemia, arthritis; congenital heart block

68
Q

how do you diagnose neonatal lupus?

A

anti-Ro in mother

69
Q

how do you treat neonatal lupus?

A

delivery if distress occurs in fetus, dexamethasone tx of mother when in utero heart block is detected; hydroxychlorquine treatment of anti-ro mother to prevent other fetuses from getting it

70
Q

how does discoid lupus present?

A

well-defined inflammatory plaques that evolve into atrophic disfiguring scars- most commonly occurs on the head

71
Q

how do you diagnose discoid lupus?

A

clinical exam and biopsy

72
Q

what does discoid lupus resemble?

A

tinea infection, psoriasis, and morphea

73
Q

what is the hallmark of scleroderma?

A

thickening and hardening of the skin; almost all patients have secondary raynaud phenomenon

74
Q

what is the first symptom typically for scleroderma and how does this differ with race?

A

white patients: raynaud’s> pigmentation changes; black patients: pigmentation changes> raynaud’s

75
Q

how would you define localized scleroderma?

A

inflammatory condition that causes hard and thickened localized patches of skin on different areas of the body; no involvement of internal organs (NOT systemic, but may affect joints or muscles in these areas)

76
Q

how does localized scleroderma present?

A

discreet patches of discolored skin induration; patches= morphea; NO raynaud’s; typically asymptomatic, but some individuals may experience itching or pain

77
Q

how do you diagnose localized scleroderma?

A

it is histologically indistinguishable from other forms of scleroderma

78
Q

how do you treat localized scleroderma?

A

quite resistant to therapy; can try phototherapy UVA or methotrexate + steroids

79
Q

how would you define limited (cutaneous) scleroderma?

A

inflammatory condition that causes hard and thickened tissues in the body (some internal organs)

80
Q

how does limited scleroderma present?

A

indolent course, raynaud’s can be one of the first symptoms; CREST syndrome; pulmonary involvement

81
Q

what does CREST stand for?

A

calcinosis cutis, raynaud’s, esophageal dysmotility, sclerodactyly, telangiectasia

82
Q

what is the pulmonary involvement seen in limited scleroderma?

A

progressive pulmonary artery hypertension (PAH)–> presents as SOB

83
Q

how do you diagnose limited scleroderma?

A

typically made in advanced disease; serology: (+) ANA and (+) anti-centromere

84
Q

how do you treat limited scleroderma?

A

no therapy to date–> manage organ system involvement; glucocorticoids show no efficacy in slowing progression and high doses may be associated with development of renal crisis

85
Q

what can be used in a patient with limited scleroderma and pulmonary hypertension?

A

phosphodiesterase type 5 inhibitor–> may improve cardiopulmonary hemodynamics

86
Q

how would you define diffuse scleroderma?

A

inflammatory condition that causes hard and thickened tissues in the body, INCLUDING internal organs

87
Q

how does diffuse scleroderma present?

A

systemic, interstitial lung disease, renal crisis

88
Q

how does interstitial lung disease present?

A

SOB, dry, course (velcro-like) crackles on auscultation

89
Q

what is a heralding feature of renal disease?

A

blood pressure elevation

90
Q

how do you diagnose diffuse scleroderma?

A

(+) anti-scl 70 aka anti (DNA) topoisomerase I; (+) anti-RNA polymerase III; (+) ANA

91
Q

how do you diagnose ILD?

A

chest x-ray or CT scan of chest: shows ground glass appearance; pulmonary function tests

92
Q

what is a main difference between scleroderma and sjogren?

A

in scleroderma, the glands are obliterated by fibrosis; in sjogren, there is destruction due to inflammatory process of immune system

93
Q

how do you diagnose pulmonary artery hypertension?

A

2D echocardiogram or right heart catheterization, which will shoe elevated pulmonary artery pressure

94
Q

those with scleroderma are at an increased risk of developing what?

A

bronchoaveolar carcinoma

95
Q

what are 3 signs of CKD?

A

proteinuria, elevation of creatinine, or HTN

96
Q

what defines renal crisis?

A

malignant hypertension, hemolytic anemia, and progressive renal insufficiency

97
Q

what endocrine disorder is common in patients with scleroderma?

A

hypothyroidism

98
Q

what MSK disease is common in patients with scleroderma?

A

carpal tunnel syndrome

99
Q

how would you define sjogren syndrome?

A

autoimmune, inflammatory destruction of exocrine glands

100
Q

how does sjogren syndrome present?

A

sicca symptoms- dryness and parotid/salivary gland enlargement

101
Q

sjogren syndrome has a strong association with what?

A

B cell non-Hodgkin lymphoma (MALT lymphoma)

102
Q

how do you diagnose sjogren syndrome?

A

serology: (+) ANA, polyclonal hypergammaglobulinemia, (+) Anti SSA/Ro, (+) Anti SSB/LA (never present without Ro); labial salivary gland biopsy

103
Q

what are the three inflammatory myopathies?

A

dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM)

104
Q

how does dermatomyositis present?

A

ages 7-15 and 30-60; weakness without sensory symptoms with characteristic skin lesions

105
Q

what are the characteristics skin lesions associated with dermatomyositis?

A

grotton’s patches/papules, heliotrope rash, periungual erythema, v-neck erythema

106
Q

patients with dermatomyositis are at increased risk of what?

A

occult malignancy

107
Q

what should you do when you make the diagnosis of dermatomyositis?

A

start looking for occult malignancy

108
Q

how doe labs appear in patients with dermatomyositis?

A

elevated CK and aldolase

109
Q

what serology is used for the diagnosis of dermatomyositis?

A

anti Jo-1

110
Q

biopsy in a patient with dermatomyositis shows what?

A

perimysial and perivascular inflammation, perifascicular atrophy

111
Q

how do you treat dermatomyositis?

A

glucocorticoids

112
Q

what is the medical term for shawl sign?

A

poikiloderma

113
Q

how does polymyositis present?

A

30-50+ years, subacute, proximal muscle weakness, no skin changes

114
Q

what do the labs look like in a patient with polymyositis?

A

elevated CK and aldolase

115
Q

what serology is used to diagnose polymyositis?

A

anti-jo1

116
Q

what does biopsy show in a patient with polymyositis?

A

endomysial inflammation with invasion of non-necrotic muscle fibers without features suggestive of another diagnosis

117
Q

how do you treat polymyositis?

A

glucocorticoids

118
Q

how does inclusion body myositis present?

A

> 40-50, M>F, white> black; weakness: finger flexion or quadriceps weakness

119
Q

what do the labs look like in a patient with inclusion body myositis?

A

mild elevation or normal CK

120
Q

what is the serology used for inclusion body myositis?

A

anti-cN1A autoantibodies

121
Q

what does biopsy show in a patient with inclusion body myositis (IBM)?

A

endomysial inflammation, rimmed vacuoles, invasion of non-necrotic muscle fibers

122
Q

how do you treat inclusion body myositis?

A

treatment is supportive

CPR II Exam 1 (13 decks)

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