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Clinical Medicine > Syndromes > Flashcards

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
Q

Dosage of prednisone for dermatomyositis/polymositis?

A

prednisone 1 mg/kg/day x 4-8 weeks (occasionally up to 3 months)

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26
Q

When do you discontinue corticosteroid treatment for dermatomyositis/polymyositis?

A

when CK level is in normal range

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27
Q

Methotrexate dosage for dermatomyositis/polymyositis

A

7.5 mg po weekly increase by 2.5 mg to 20 mg po weekly as needed (take with folic acid 1 mg)

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28
Q

what is fibromyalgia?

A

chronic widespread pain and tenderness

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29
Q

What’s the patho of fibromyalgia?

A

abnormal central sensory processing within the CNS; amplification of peripheral sensory stimuli; descending inhibitory control deficit in midbrain; persistent neuronal hyperexcitability

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30
Q

Etiology of fibromylagia

A

unknown maybe hormones, sleep disorders, depression, viral infections, rare complication of hypothyroidism, RA, or sleep apnea

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31
Q

Does fibromylalgia impact men or women more often?

A

women

32
Q

Age range for women with fibromyalgia?

A

20-50; increases with age

33
Q

S/S of fibromyalgia

A

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
Q

Labs for fibromyalgia

A

CBC with diff, BMP, thyroid stimulating hormone, ESR, CRP, ANA, RF, CPK

35
Q

Treatment for fibromyalgia

A

education, counseling/behavior modification, pain, treat causes of fatigue/sleep difficulties, eliminate stressors, deconditioning (PT/OT), maybe treat endocrine dysfunction

36
Q

Medication treatments for fibromyalgia

A

analgesics (acetaminophen, tramadol); muscle relaxants (cyclobenzaprine); anti-anxiety; antidepressants; anticonvulsants

37
Q

Tramadol dosage for fibromyalgia

A

50 mg 1 6-8 hours PRN (short term)

38
Q

Cyclobenzaprine dosage for fibromylagia

A

5 mg po q8 hours PRN

39
Q

ex of anti-anxiety meds for fibromyalgia

A

alprazolam, buspiron, clonazepam, zolpidem, zaleplon

40
Q

ex of antidepressants for fibromyalgia

A

amitriptyline, duloxetine, venlafaxine

41
Q

Anticonvulsant ex for treatment of fibromyalgia

A

gabapentin, pregabalin

42
Q

What’s CFS?

A

myalgic encephalomyelitis

43
Q

Cause of CFS?

A

viral association? EBV, coxsackievirus B

44
Q

Are men or women more commonly impacted by CFS?

A

women

45
Q

CM of CFS

A

sudden onset abrupt, debilitating fatigue (impacts ADL, relationships, work funcitons); possible flu-like prodrome; generalized MSK pain, cognitive dysfunction, IBS, nonrestorative sleep

46
Q

How long does the fatigue have to be to be diagnosed with CFS?

A

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
Q

Outside of fatigue, what other criteria can be met for CFS (4/8)

A

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
Q

What disorders cause fatigue?

A

heart disease, thyroid disease, chronic anemia, liver and renal disease, psychiatric illness, fibromyalgia

49
Q

Work up for CFS?

A

r/o

50
Q

Treatment of CFS?

A

supportive and symptomatic

51
Q

What is complex regional pain syndrome?

A

affects the extremities (pain, swelling, and vasomotor dysfunction) following trauma/surgery/soft tissue disorder/immobilization

52
Q

Who does complex regional pain syndrome impact more?

A

women

53
Q

Age of average complex regional pain syndrome?

A

30-60 with mean age at 40

54
Q

CM of complex regional pain syndrome

A

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
Q

How many extremities does CRPS impact?

A

usually only one

56
Q

what are the three clinical stages of CRPS?

A

acute, subacute, chronic

57
Q

duration of acute CRPS

A

3 months

58
Q

describe acute CRPS

A

burning pain, swelling, redness, hyperhidrosis, coolness to touch, functional limitations, bone demineralization

59
Q

duration of subacute CRPS

A

9 months

60
Q

describe subacute CRPS

A

severe pain, fixed edema, pallor and cyanosis, dry skin, loss of function (joint fibrosis)

61
Q

Duration of chronic CRPS

A

1 year after onset

62
Q

describe chronic CRPS

A

variable pain, decreased edema, dry/pale/cool/shiny skin, osteoporosis, loss of function (fibrosis)

63
Q

how long does CRPS last?

A

chronic can last for years or be permanent

64
Q

labs for CRPS

A

nothing specific

65
Q

imaging for CRPS

A

pain films (bone demineralization), triple phase bone scan (increased uptake in articular and periarticular structures)

66
Q

treatment of CRPS

A

patient education, meds (NSAIDs/corticosteroids), sympathetic nerve block, PT/OT, surgical last resort

67
Q

surgical treatment of CRPS

A

upper thoracic/lumbar symphathectomy, chemical sympathectomy, spinal cord stimulator implantation

68
Q

Genetic dominance of Marfan

A

AD

69
Q

S/S of Marfan

A

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
Q

What’s Ehlers-Danlos syndrome?

A

heterogenous disorder of connective tissue; affects structure, production, or processing of collagen

71
Q

Hallmarks of Ehlers-Danlos

A

tissue fragility, joint hypermobility, skin hyperextensibility

72
Q

Ehlers-Danlos s/s

A

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
Q

treatment of ehlers-danlos syndrome

A

manifestations of the syndrome, care with suturing, prophylactic beta blocker to protect vessels maybe

74
Q

Osteogenesis imperfecta

A

hereditary osteopenia due to defects in bony protein matrix

75
Q

s/s of osteogenesis imperfecta

A

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
Q

Tx of OI

A

Risedronate to increase bone mineral density

Clinical Medicine (13 decks)

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