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Syndromes Flashcards

1
Q

What are polymyositis and dermatomyositis?

A

group idiopathic inflammatory myopathies

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2
Q

Dermatomyositis can be associated with?

A

malignancy

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3
Q

Does polymyositis and dermatomyositis impact men or women more?

A

women

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4
Q

Patho of dermatomyositis?

A

humoral attack to muscle capillaries and small arterioles; activation of C3 forms C3bNEO and MAC; B cells and Cd4 cells activated; destroys capillaries; causes microinfarction in the muscles

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5
Q

Patho of polymositis

A

T-cell mediated cytotoxic process against muscle antigens

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6
Q

Genetic component of polymyositis and dermatomyositis?

A

HLA types– DR3, DR5, DR7

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7
Q

Immunologic etiology of polymyositis and dermatomyositis

A

abnormal t-cell activity

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8
Q

Infectious etiology of polymyositis and dermatomyositis

A

viruses (coxsackievirus, parvovirus, HIV), toxoplasma species, borrelia species

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9
Q

Drug etiology of polymyositis and dermatomyositis

A

statins, interferon, quinidine

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10
Q

CM of polymyositis and dermatomyositis

A

skin is the initial manifestation in dermatomyositis only; proximal muscle weakness (pelvic girdle, upper body weakness, neck extensor muscles); difficulty climbing stairs/walking/standing up from seated position/combing hair/lifting things; possible muscle tenderness to palpation

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11
Q

Systemic manifestations of polymyositis and dermatomyositis

A

arthralgias and arthritis (knees, wrist, hands), fatigue, weight loss, fever, Raynaud’s, dysphagia, reflux esophagitis, constipation, exertional dypsnea, interstitial lung disease, rhythm disturbances, conduction defects, CHF, pericarditis

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12
Q

PE of dermatomyositis

A

possible photo-sensitivity over face, chest, hands; Gottron’s papules; rash in V neck distribution; calcinosis

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13
Q

Calcinosis is seen in?

A

Dermatomyositis and Scleroderma

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14
Q

What’s Gottron’s papules?

A

raised, scaly palpable lesions on extensor surfaces; MCP, PIP, elbows, upper eyelids

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15
Q

Labs for dermatomyositis/polymyositis

A

CBC (possible leukocytosis or thrombocytosis), ESR (elevated), creatine kinase (elevated), AST/LDH (may be abnormal), autoantibody studies: ANA (positive), anti-jo-1 (polymyositis), anti-Mi2 and anti-MDA5–dermatomyositis

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16
Q

Which autoantibody study is specific to polymyositis?

A

anti-jo-1

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17
Q

which autoantibody study is specific to dermatomyositis?

A

anti-Mi2 and anti-MDA5

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18
Q

Imaging for dermatomyositis/polymyositis

A

MRI to evaluate for amount of muscle involvement; EMG (abnormal; not done as frequently as MRI); muscle biopsy

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19
Q

What’s necessary for definitive dermatomyositis/polymyositis diagnosis?

A

muscle biopsy

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20
Q

What are important non-diagnostic testing for dermatomyositis?

A

colonoscopy, pap smear, mammogram, CA-125, CA 19-9

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21
Q

Diagnostic criteria of dermatomyositis

A

[Gottron’s papules or muscle biopsy finding of perifasicular atrophy] OR all of the following are present: suggestive skin involvement, proximal weakness (subacute/chronic), muscle biopsy or skin biopsy (perimysial or perivascular inflammation)

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22
Q

Diagnostic criteria of polymyositis?

A

all of the following: subacute/chronic proximal weakness, elevated serum creatine kinase, muscle biopsy (endomysial inflammation), response to immunotherapy or exclusion of limb-girdle muscular dystrophies

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23
Q

Treatment of dermatomyositis/polymyositis?

A

high protein diet, daily exercise, avoid UV exposure/use sunscreen, corticosteroids (topical), hydroxychloroquine, methotrexate

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24
Q

What’s the first line treatment (Drug) for dermatomyositis/polymositis?

A

corticosteroids

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25
Dosage of prednisone for dermatomyositis/polymositis?
prednisone 1 mg/kg/day x 4-8 weeks (occasionally up to 3 months)
26
When do you discontinue corticosteroid treatment for dermatomyositis/polymyositis?
when CK level is in normal range
27
Methotrexate dosage for dermatomyositis/polymyositis
7.5 mg po weekly increase by 2.5 mg to 20 mg po weekly as needed (take with folic acid 1 mg)
28
what is fibromyalgia?
chronic widespread pain and tenderness
29
What's the patho of fibromyalgia?
abnormal central sensory processing within the CNS; amplification of peripheral sensory stimuli; descending inhibitory control deficit in midbrain; persistent neuronal hyperexcitability
30
Etiology of fibromylagia
unknown maybe hormones, sleep disorders, depression, viral infections, rare complication of hypothyroidism, RA, or sleep apnea
31
Does fibromylalgia impact men or women more often?
women
32
Age range for women with fibromyalgia?
20-50; increases with age
33
S/S of fibromyalgia
widespread pain and stiffness (neck, shoulders, low back, hips), fatigue, disordered sleep (non-restorative sleep, restless leg syndrome), cognitive dysfunction, depression, chronic HA, IBS, TMJ pain, chronic pelvic pain, overactive bladder syndrome
34
Labs for fibromyalgia
CBC with diff, BMP, thyroid stimulating hormone, ESR, CRP, ANA, RF, CPK
35
Treatment for fibromyalgia
education, counseling/behavior modification, pain, treat causes of fatigue/sleep difficulties, eliminate stressors, deconditioning (PT/OT), maybe treat endocrine dysfunction
36
Medication treatments for fibromyalgia
analgesics (acetaminophen, tramadol); muscle relaxants (cyclobenzaprine); anti-anxiety; antidepressants; anticonvulsants
37
Tramadol dosage for fibromyalgia
50 mg 1 6-8 hours PRN (short term)
38
Cyclobenzaprine dosage for fibromylagia
5 mg po q8 hours PRN
39
ex of anti-anxiety meds for fibromyalgia
alprazolam, buspiron, clonazepam, zolpidem, zaleplon
40
ex of antidepressants for fibromyalgia
amitriptyline, duloxetine, venlafaxine
41
Anticonvulsant ex for treatment of fibromyalgia
gabapentin, pregabalin
42
What's CFS?
myalgic encephalomyelitis
43
Cause of CFS?
viral association? EBV, coxsackievirus B
44
Are men or women more commonly impacted by CFS?
women
45
CM of CFS
sudden onset abrupt, debilitating fatigue (impacts ADL, relationships, work funcitons); possible flu-like prodrome; generalized MSK pain, cognitive dysfunction, IBS, nonrestorative sleep
46
How long does the fatigue have to be to be diagnosed with CFS?
persistent chronic fatigue >6 months or intermittent, unexplained chronic fatigue (relapses and definite start) and not the result of exertion and is not relieved by rest
47
Outside of fatigue, what other criteria can be met for CFS (4/8)
recently impaired memory/concentration, pain on swallowing, painful axillary/cervical lymph nodes, muscle pains, joint pain without swelling, HA, sleep that does not improve after rest, post-exertional discomfort lasting >24 hours
48
What disorders cause fatigue?
heart disease, thyroid disease, chronic anemia, liver and renal disease, psychiatric illness, fibromyalgia
49
Work up for CFS?
r/o
50
Treatment of CFS?
supportive and symptomatic
51
What is complex regional pain syndrome?
affects the extremities (pain, swelling, and vasomotor dysfunction) following trauma/surgery/soft tissue disorder/immobilization
52
Who does complex regional pain syndrome impact more?
women
53
Age of average complex regional pain syndrome?
30-60 with mean age at 40
54
CM of complex regional pain syndrome
pain out of proportion, edema (disproportionate), redness/cyanosis/pale, stiffness/decreased ROM, muscle weakness/tremors, excessive sweating/dryness/shiny/wrinkled/temp changes, flexion contractures, frozen shoulder, joint fibrosis
55
How many extremities does CRPS impact?
usually only one
56
what are the three clinical stages of CRPS?
acute, subacute, chronic
57
duration of acute CRPS
3 months
58
describe acute CRPS
burning pain, swelling, redness, hyperhidrosis, coolness to touch, functional limitations, bone demineralization
59
duration of subacute CRPS
9 months
60
describe subacute CRPS
severe pain, fixed edema, pallor and cyanosis, dry skin, loss of function (joint fibrosis)
61
Duration of chronic CRPS
1 year after onset
62
describe chronic CRPS
variable pain, decreased edema, dry/pale/cool/shiny skin, osteoporosis, loss of function (fibrosis)
63
how long does CRPS last?
chronic can last for years or be permanent
64
labs for CRPS
nothing specific
65
imaging for CRPS
pain films (bone demineralization), triple phase bone scan (increased uptake in articular and periarticular structures)
66
treatment of CRPS
patient education, meds (NSAIDs/corticosteroids), sympathetic nerve block, PT/OT, surgical last resort
67
surgical treatment of CRPS
upper thoracic/lumbar symphathectomy, chemical sympathectomy, spinal cord stimulator implantation
68
Genetic dominance of Marfan
AD
69
S/S of Marfan
lens displacement, risk of retinal detachment, increased limb and finger length/sternal deformity, ligamentous laxity, arched palate, crowded dentition, malocclusion, MVP, aortic root dilation, spontaneous pneumothorax, sleep apnea, striae atrophy, cystic organs
70
What's Ehlers-Danlos syndrome?
heterogenous disorder of connective tissue; affects structure, production, or processing of collagen
71
Hallmarks of Ehlers-Danlos
tissue fragility, joint hypermobility, skin hyperextensibility
72
Ehlers-Danlos s/s
joint hypermobility, skin atrophy/velvety skin, medium and large vessel rupture, easily bruises, organ rupture (uterus, large bowel, pneumothorax), premature rupture of fetal membranes
73
treatment of ehlers-danlos syndrome
manifestations of the syndrome, care with suturing, prophylactic beta blocker to protect vessels maybe
74
Osteogenesis imperfecta
hereditary osteopenia due to defects in bony protein matrix
75
s/s of osteogenesis imperfecta
recurrent fractures with deformity, impaired mobility, short stature, restrictive lung disease (vertebral column deformity/rib fractures), compression of brain stem, abnormal teeth, BLUE SCLERA
76
Tx of OI
Risedronate to increase bone mineral density

Clinical Medicine (13 decks)

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