MSK and Connective Tissue Disorders II Flashcards

1
Q

who as the most susceptible to lupus?

A

Americans of African descent

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2
Q

lupus is a ______ tissue disease

A

connective

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3
Q

Congenital deficiencies of what are associated w/ increased incidence of SLE?

A

C1, C2, C4

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4
Q

when is onset of lupus most common?

A

2nd and 3rd decades

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5
Q

people with lupus have a higher risk of what?

A

premature death

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6
Q

lupus is a ____ system problem

A

profound immune

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7
Q

In lupus there is an uncontrolled production of what?

A

autoantibodies and immune complexes

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8
Q

2 ways tissue injuries occur in lupus?

A

formation of immune complexes (ex- glomerulonephritis)

damage/ destruction of specific cells (ex- generation of C3 and C5) causing inflammation,

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9
Q

what hormone plays a factor in SLE?

A

estrogen

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10
Q

when are exacerbation of SLE common?

A

pregnancy and OCP meds

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11
Q

male with what condition have an increased incidence of SLE.

A

Klinefelter’s

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12
Q

what drugs can cause drug induced lupus?

A
procainamide
hydralazine
isoniazid
methyldopa
quinidine
chlorpromazine
phenothiazines
OCPs
anticonvulsants
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13
Q

if a patient has a drug induced lupus will that ANA be positive?

A

yes, it can be

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14
Q

if you suspect drug induced lupus what must you do?

A

Stop drug, monitor symptoms and then repeat ANA

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15
Q

what are the three main symptoms of lupus

A

arthritis (joint inflammation)
arthralgia
fever

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16
Q

what syndrome if common in SLE?

A

Raynaud’s syndrome

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17
Q

a hx of what is common in women with SLE.

A

spontaneous abortions

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18
Q

what are skin signs common with SLE

A

vasculitis
purpura
periungal erythema

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19
Q

what often leads to deformities and contractures in SLE?

A

chronic arthritis

tensosynovitis

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20
Q

the involvement of what organ with SLE has the worst prognosis

A

renal

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21
Q

what are renal symptoms of SLE

A

nephrotic symptoms

renal failure

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22
Q

what are some CNS symptoms seen with SLE

A
epilepsy
depression, dementia, psychosis
cranial nerve lesions
hemiplegia, transverse myelitis
chorea, ataxia
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23
Q

what are ocular manifestations of SLE?

A

keratoconjunctivitis
episcleritis
retinal vasculitis
retinal exudates

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24
Q

what are abdominal manifestations of SLE?

A

peritonitis
splenitis
pancreatitis
GI ulcers

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25
Q

what do you need for a lupus diagnosis?

A

Positive ANA and four crieteria

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26
Q

what are SLE criteria for diagnosis

A
malar rash
photosensitivity
arthritis
serositis (hearts/ lungs)
renal dz (proteinuria, cellular casts)
hematologic disorders (hemolytic anemia, leucopenia, leukocytosis, thrombocytopenia)
immune disorders (include false + syphilis)
neuro dz (seizures, psychosis)
discoid rash
oral ulcers
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27
Q

people with SLE can present with spontaneous what?

A

DVTs

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28
Q

what labs should you get for SLE?

A
ANA
CBC
BUn
creatinine
UA
ESR
Serum complement (C3 and/or C4)
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29
Q

what can be used to monitor the progression of SLE?

A

antibodies to Smith antigen
dsDNA
depressed serum complement

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30
Q

Tx for lupus

A

NSAIDs
regular exercise and sun protection
antimalarial
corticosteroids

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31
Q

what antimalarials are used for SLE?

A

Hydroxychloroquine or quinacrine

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32
Q

what is the best combo for corticosteroids for SLE to prevent renal failure and tx symptoms?

A

Prednisone and cyclophosphamide

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33
Q

drug for SLE that works well for arthritis, rashes, serositis, and constitutional symptoms

A

methotrexate

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34
Q

long term use of ________ carries a risk of infertility in females >25 and increase malignancy risk

A

cyclophosphamide

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35
Q

what s late mortality w/ SLE usually due to?

A

secondary CAD or CVA

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36
Q

what are the most common infections in SLE?

A

pneumocystis carinii

candida albicans

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37
Q

most SLE infection respond to what?

A

steroid therapy

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38
Q

Overlapping clinical features suggestive of SLE, progressive sclerosis & polymyositis

A

mixed connective tissue disorders

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39
Q

will mixed connective tissue diseases have a high ANA titer (>1:80)

A

Yes

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40
Q

what diseases are included in mixed connective tissue diseases

A

Sjogrens
scleroderma
vasculitis

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41
Q

Autoimmune disorder causing destruction of salivary and lacrimal glands (exocrine glands)

A

Sjogren’s syndrome

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42
Q

can sjogren’s be a complication of a prexxsisting disease?

A

Yes, in RA, SLE, polymyositis, scleroderma

43
Q

who is sjogren’s most common in?

A

middle-aged females

44
Q

what are clinical features of sjogren’s syndrome?

A
dry mouth (xerostomia)
dry eyes (xerophthalmia or keratoconjunctivitis sicca)
enlargement of parotid glands
45
Q

what will labs look like w/ connective tissue disorders

A

Don’t quite make sense

some positive, some negative

46
Q

What test evaluates tear secretions for Sjogren’s

A

Schrimer’s test

47
Q

how do you do schirmer’s test

A

– wetting of less than 5 mm of filter paper placed in lower eyelid for 5 min is considered +
(for sjogren’s)

48
Q

tx for sjogren’s syndrome

A

artificial tears and saliva
protect the eyes
increase PO intake
ocular and vaginal lubricants

49
Q

are steroid indicated in Sjogren’s?

A

rarely

50
Q

What drugs are helpful for Sjogren’s

A

NSAIDS (inflammation)
corticosteroids for huge flare up
ACEI (if HTN)

51
Q

Tx for Raynaud’s

A

Nifedipine (CCB)
prostacyclin
protect limbs from cold

52
Q

when is sjogren’s prognosis worse

A

late onset

widespread skin, renal, pulmonary, cardiac inolvement

53
Q

what do people of sjogren’s usually die of

A

aneurysms or renal issues

54
Q

what are the two types of scleroderma?

A

diffuse (systemic)

limited (skin of face, neck, elbows, knees)

55
Q

where does scleroderma most often begin?

A

fingers and hands

56
Q

What do most people with scleroderma typically have?

A

Raynaud’s phenomenon

57
Q

what is a common presentation of scleroderma

A

skin changes
polyarthralgias
esophageal dysunfction

58
Q

what does CREST syndrome stand for?

A
Calcinosis
Raynaud's
esophageal dysfunction
sclerodactyly
telangiectasia's
59
Q

is ANA typically positive in scleroderma?

A

Yes

60
Q

what must you monitor w/ scleroderma

A

development of HTN

look for kidney problems

61
Q

Multi-system, autoimmune disease of unknown cause

Vasculopathy & excess collagen deposits are characteristic

A

systemic sclerosis

62
Q

what does systemic sclerosis lead to

A

fibrosis

degenerative changes of skin and internal organs

63
Q

what are early manifestations of systemic sclerosis

A
raynaud's phenomenon 
skin calcinosis (scleroderma)
64
Q

who is systemic sclerosis common in?

A

females ages 30-60

65
Q

what are early manifestations of systemic sclerosis

A
wrist pain
wheezing
skin- abnormally light/dark pigment
joint pain
hair loss, alopecia
eye burning, itching, discharge
arthralgia
66
Q

what is a unique component of systemic sclerosis

A

non-pitting edema

sausage swelling of fingers

67
Q

what are some GI problems w/ systemic sclerosis

A
reflux/ hiatal hernia
loss of normal peristalsis
dilation of large and small bowel
pain, constipation, diarrhea
primary biliary cirrhosis is associated
68
Q

what does patients w/ systemic sclerosis die of?

A

constrictive pericarditis

69
Q

if someone has pulmonary fibrosis/ dz out of no where what should you look at?

A

connective tissue disorders

70
Q

Tx for symptoms of systemic sclerosis

A

PPI for GERD
ACEI for renal
CCB for Raynaud’s
immunosupressive drugs for pulmonary HTN

71
Q

what are complications from glucocorticoids?

A
cushing's syndrome
osteoporosis
DM
Infection
IMmunosuppression
72
Q

What are ADRs from cyclophosphamide?

A
Pancytopenia
mucositis
alopecia
hemorrhagic cystitis
TC carcinoma
sterility
73
Q

what are ADRs of azathioprine?

A

leukopenia
anemia
risk of infection
possible hematologic malignancy

74
Q

what are ADRS of methotrexate

A

Teratogenic
liver fibrosis
infection

75
Q

ADRs of vasodilators

A

HPOTN

constipation

76
Q

ADRs of antimalarials

A

retinal toxicity

77
Q

what muscles does inflammatory myopathy usually affect?

A

proximal muscles

78
Q

What test is abnormal in inflammatory myopathy?

A

EMG

79
Q

what are the three inflammatory myopathies

A

dermatomyositis
polymyositis
inclusion body myositis

80
Q

what makes dermatomyositis unique?

A

rash
good response to corticosteroids
increased serum creatinine cleranace
perifascular atrophy (on muscle biopsy)

81
Q

Inflammation of large, medium, or small blood vessels leading to end-organ or necrosis/tissue damage

A

Systemic vasculitis

82
Q

Acute systemic disorder of childhood
Resembles viral exanthem
Boys affected 3x more than girls

A

Kawasaki’s Disease

83
Q

what complications are associated w/ Kawasaki’s Disease?

A
coronary aneurysms
coronary artery dilatation
MI
pericarditis
effusion
cardiac failure
84
Q

Tx for Kawasaki’s Disease

A

ASA 5mg/kg/day

high dos IVIG

85
Q

should people with kawasaki’s be given steroids?

A

No, can increase coronary dilatation

86
Q

tx for vasculitis

A

corticosteroids or cytotoxic drugs

plasmapheresis

87
Q

complications of vasculitis

A
coronary aneursysms
coronary artery dilatation
MI
pericarditis w/ effusion
cardiac failure
88
Q

what three things is temporal arteritis associated with?

A

steroids
blindness
biopsy

89
Q

Pain and stiffness in neck, shoulder, upper arms, pelvic girdles and thighs + constitutional symptoms (fever, wt loss, fatigue, depression). Usually >50

A

polymyalgia rheumatica

90
Q

tx for polymyalgia rheumatica

A

steroids

91
Q

what is also often seen w/ polymyalgia rheumatica

A

temporal arteritis

92
Q

Presents with scalp tenderness, jaw claudication, HA, temporal artery tenderness
May lead to vision loss

A

temporal arteritis

93
Q

when is stiffness more severe w/ polymyalgia rheumatica

A

after rest and in AM

94
Q

what lab test do you do for polymyalgia rheumatica?

A

ESR markedly elevated (>50)

95
Q

Tx for polymyalgia rheumatica

A

low-dose corticosteroid therapy (PO)
may be required for 2 years, must slowly taper off
higher doses needed when temporal arteritis present

96
Q

fibromyalgia is a diagnosis of _______

A

exclusion

97
Q

clinical features of fibromyalgia

A

Non-articular musculoskeletal aches, pains, fatigue, sleep disturbance, and multiple tender points on exam (is a chart available for practice)

98
Q

there is a strong _________ component in fibromyalgia

A

psych

99
Q

what do you need to exclude to make a dx of fibromyalgia?

A

thyroid disease
hep C
vit D deficiency
abnormal T-cell subsets (not diagnostic)

100
Q

do you give narcotics for fibromyalgia?

A

No

101
Q

what drugs may be helpful for fibromyalgia?

A

SSRIs
SNRIs
TCAs
Lyrica (only FDA approved)

102
Q

what labs are helpful to r/o other pathology before dx fibromyalgia

A
x-rays around joints
CBC
ESR
RA factor
ANA
CPK helpful (elevated in muscle destruction)
103
Q

what will be abnormal in a sleep study in a person w/ fibromyalgia

A

disturbance of stage IV non-REM on EEG