MSK and Connective Tissue Disorders II Flashcards

(103 cards)

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
what do you need for a lupus diagnosis?
Positive ANA and four crieteria
26
what are SLE criteria for diagnosis
``` 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 ```
27
people with SLE can present with spontaneous what?
DVTs
28
what labs should you get for SLE?
``` ANA CBC BUn creatinine UA ESR Serum complement (C3 and/or C4) ```
29
what can be used to monitor the progression of SLE?
antibodies to Smith antigen dsDNA depressed serum complement
30
Tx for lupus
NSAIDs regular exercise and sun protection antimalarial corticosteroids
31
what antimalarials are used for SLE?
Hydroxychloroquine or quinacrine
32
what is the best combo for corticosteroids for SLE to prevent renal failure and tx symptoms?
Prednisone and cyclophosphamide
33
drug for SLE that works well for arthritis, rashes, serositis, and constitutional symptoms
methotrexate
34
long term use of ________ carries a risk of infertility in females >25 and increase malignancy risk
cyclophosphamide
35
what s late mortality w/ SLE usually due to?
secondary CAD or CVA
36
what are the most common infections in SLE?
pneumocystis carinii | candida albicans
37
most SLE infection respond to what?
steroid therapy
38
Overlapping clinical features suggestive of SLE, progressive sclerosis & polymyositis
mixed connective tissue disorders
39
will mixed connective tissue diseases have a high ANA titer (>1:80)
Yes
40
what diseases are included in mixed connective tissue diseases
Sjogrens scleroderma vasculitis
41
Autoimmune disorder causing destruction of salivary and lacrimal glands (exocrine glands)
Sjogren's syndrome
42
can sjogren's be a complication of a prexxsisting disease?
Yes, in RA, SLE, polymyositis, scleroderma
43
who is sjogren's most common in?
middle-aged females
44
what are clinical features of sjogren's syndrome?
``` dry mouth (xerostomia) dry eyes (xerophthalmia or keratoconjunctivitis sicca) enlargement of parotid glands ```
45
what will labs look like w/ connective tissue disorders
Don't quite make sense | some positive, some negative
46
What test evaluates tear secretions for Sjogren's
Schrimer's test
47
how do you do schirmer's test
– wetting of less than 5 mm of filter paper placed in lower eyelid for 5 min is considered + (for sjogren's)
48
tx for sjogren's syndrome
artificial tears and saliva protect the eyes increase PO intake ocular and vaginal lubricants
49
are steroid indicated in Sjogren's?
rarely
50
What drugs are helpful for Sjogren's
NSAIDS (inflammation) corticosteroids for huge flare up ACEI (if HTN)
51
Tx for Raynaud's
Nifedipine (CCB) prostacyclin protect limbs from cold
52
when is sjogren's prognosis worse
late onset | widespread skin, renal, pulmonary, cardiac inolvement
53
what do people of sjogren's usually die of
aneurysms or renal issues
54
what are the two types of scleroderma?
diffuse (systemic) | limited (skin of face, neck, elbows, knees)
55
where does scleroderma most often begin?
fingers and hands
56
What do most people with scleroderma typically have?
Raynaud's phenomenon
57
what is a common presentation of scleroderma
skin changes polyarthralgias esophageal dysunfction
58
what does CREST syndrome stand for?
``` Calcinosis Raynaud's esophageal dysfunction sclerodactyly telangiectasia's ```
59
is ANA typically positive in scleroderma?
Yes
60
what must you monitor w/ scleroderma
development of HTN | look for kidney problems
61
Multi-system, autoimmune disease of unknown cause | Vasculopathy & excess collagen deposits are characteristic
systemic sclerosis
62
what does systemic sclerosis lead to
fibrosis | degenerative changes of skin and internal organs
63
what are early manifestations of systemic sclerosis
``` raynaud's phenomenon skin calcinosis (scleroderma) ```
64
who is systemic sclerosis common in?
females ages 30-60
65
what are early manifestations of systemic sclerosis
``` wrist pain wheezing skin- abnormally light/dark pigment joint pain hair loss, alopecia eye burning, itching, discharge arthralgia ```
66
what is a unique component of systemic sclerosis
non-pitting edema | sausage swelling of fingers
67
what are some GI problems w/ systemic sclerosis
``` reflux/ hiatal hernia loss of normal peristalsis dilation of large and small bowel pain, constipation, diarrhea primary biliary cirrhosis is associated ```
68
what does patients w/ systemic sclerosis die of?
constrictive pericarditis
69
if someone has pulmonary fibrosis/ dz out of no where what should you look at?
connective tissue disorders
70
Tx for symptoms of systemic sclerosis
PPI for GERD ACEI for renal CCB for Raynaud's immunosupressive drugs for pulmonary HTN
71
what are complications from glucocorticoids?
``` cushing's syndrome osteoporosis DM Infection IMmunosuppression ```
72
What are ADRs from cyclophosphamide?
``` Pancytopenia mucositis alopecia hemorrhagic cystitis TC carcinoma sterility ```
73
what are ADRs of azathioprine?
leukopenia anemia risk of infection possible hematologic malignancy
74
what are ADRS of methotrexate
Teratogenic liver fibrosis infection
75
ADRs of vasodilators
HPOTN | constipation
76
ADRs of antimalarials
retinal toxicity
77
what muscles does inflammatory myopathy usually affect?
proximal muscles
78
What test is abnormal in inflammatory myopathy?
EMG
79
what are the three inflammatory myopathies
dermatomyositis polymyositis inclusion body myositis
80
what makes dermatomyositis unique?
rash good response to corticosteroids increased serum creatinine cleranace perifascular atrophy (on muscle biopsy)
81
Inflammation of large, medium, or small blood vessels leading to end-organ or necrosis/tissue damage
Systemic vasculitis
82
Acute systemic disorder of childhood Resembles viral exanthem Boys affected 3x more than girls
Kawasaki's Disease
83
what complications are associated w/ Kawasaki's Disease?
``` coronary aneurysms coronary artery dilatation MI pericarditis effusion cardiac failure ```
84
Tx for Kawasaki's Disease
ASA 5mg/kg/day | high dos IVIG
85
should people with kawasaki's be given steroids?
No, can increase coronary dilatation
86
tx for vasculitis
corticosteroids or cytotoxic drugs | plasmapheresis
87
complications of vasculitis
``` coronary aneursysms coronary artery dilatation MI pericarditis w/ effusion cardiac failure ```
88
what three things is temporal arteritis associated with?
steroids blindness biopsy
89
Pain and stiffness in neck, shoulder, upper arms, pelvic girdles and thighs + constitutional symptoms (fever, wt loss, fatigue, depression). Usually >50
polymyalgia rheumatica
90
tx for polymyalgia rheumatica
steroids
91
what is also often seen w/ polymyalgia rheumatica
temporal arteritis
92
Presents with scalp tenderness, jaw claudication, HA, temporal artery tenderness May lead to vision loss
temporal arteritis
93
when is stiffness more severe w/ polymyalgia rheumatica
after rest and in AM
94
what lab test do you do for polymyalgia rheumatica?
ESR markedly elevated (>50)
95
Tx for polymyalgia rheumatica
low-dose corticosteroid therapy (PO) may be required for 2 years, must slowly taper off higher doses needed when temporal arteritis present
96
fibromyalgia is a diagnosis of _______
exclusion
97
clinical features of fibromyalgia
Non-articular musculoskeletal aches, pains, fatigue, sleep disturbance, and multiple tender points on exam (is a chart available for practice)
98
there is a strong _________ component in fibromyalgia
psych
99
what do you need to exclude to make a dx of fibromyalgia?
thyroid disease hep C vit D deficiency abnormal T-cell subsets (not diagnostic)
100
do you give narcotics for fibromyalgia?
No
101
what drugs may be helpful for fibromyalgia?
SSRIs SNRIs TCAs Lyrica (only FDA approved)
102
what labs are helpful to r/o other pathology before dx fibromyalgia
``` x-rays around joints CBC ESR RA factor ANA CPK helpful (elevated in muscle destruction) ```
103
what will be abnormal in a sleep study in a person w/ fibromyalgia
disturbance of stage IV non-REM on EEG