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Musculoskeletal and Connective Tissue > Scleroderma - muscle diseases - sarcoidosis > Flashcards

Scleroderma - muscle diseases - sarcoidosis Flashcards

1
Q

scleroderma is a

A

autoimmune disease characterised by activation of fibroblasts and deposition of collagen
aka: systemic sclerosis

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2
Q

characteristics of scleroderma - triad

A
  1. autoimmunity
  2. noninflammatory vasculopathy
  3. collagen deposition with fibrosis
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3
Q

scleroderma causes sclerosis of (systems)

A
  1. skin
  2. renal
  3. pulmonary
  4. cardiovascular
  5. GI system
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4
Q

scleroderma - sex

A

75% females

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5
Q

systemic scleroderma is divided into

A
  1. diffuse scleroderma

2. limited scleroderma

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6
Q

localized scleroderma involves

A

only skin

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7
Q

systemic scleroderma involves

A

both skin and visceral organs

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8
Q

scleroderma - manifestation of skin (appearance)

A

puffy , taut skin without wrinkles, fingertip pitting

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9
Q

diffuse scleroderma - clinical characteristics

A
  1. widespread skin involvement
  2. rapid progression
  3. early visceral involvement
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10
Q

diffuse scleroderma is associated with

A

anti-Scl 70 antibody (anti-DNA topoisomerase I antibody)

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11
Q

anti-Scl 70 antibody is

A

anti-DNA topoisomerase I antibody

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12
Q

Limited sclerodermia - clinical manifestation

A
  • limited skin involvement (fingers and face)

- CREST: Calcinosis, Raynaud phenomenon, Esophangeal dysmotility, Sclerodactily, Talangiectasia

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13
Q

CREST - manifestation

A

Calcinosis, Raynaud phenomenon, Esophangeal dysmotility, Sclerodactily, Talangiectasia

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14
Q

Calcinosis is the

A

formation of calcium deposits in any soft tissue.

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15
Q

telangiectasia is a condition characterized by

A

dilatation of the capillaries causing them to appear as small red or purple clusters (skin and organs)

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16
Q

Limited sclerodermia is associated with

A

anti-centromere antibodies

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17
Q

MCC of death in scleroderma

A

pulmonary involvement

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18
Q

Limited vs systemic sclerodermia according the course

A

Limited has more benign clinical course

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19
Q

Myositis ossificans is a

A

heterotopic ossification (metaplasia into bone) of skeltal muscle following muscular trauma

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20
Q

Myositis ossificans in greek

A

οστεοποιός μυοσίτιδα

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21
Q

Myositis ossificans - metaplasia

A

skeltal muscle into bone

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22
Q

Myositis ossificans - MC area of the body

A

upper and lower extremity

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23
Q

Myositis ossificans may present as

A
  • a suspicious mass at site of known trauma

- incidental finding on radiography

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24
Q

2 neuromascular junction diseases

A
  1. myasthenia gravis

2. Lambert-Eaton myasthenic syndrome

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25
Q

MC neuromascular junction disorder

A

Myasthenia gravis

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26
Q

myasthenia gravis vsLambert-Eaton according frequency

A
  1. myasthenia gravis –> MC uromascular junction disorder

2. Lambert-Eaton –> uncommon

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27
Q

pathophysiology of myasthenia gravis

A

autoantibodies to postsynaptic ACH receptor

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28
Q

pathophysiology of Lambert-Eaton myasthenic syndrome

A

autoantibodies to presynaptic Ca2+ channel –> decreased ACH release

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29
Q

myasthenia gravis is associated with

A
  1. thymoma

2. thymic hyperplasia

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30
Q

Lambert-Eaton myasthenic syndrome is associated with

A

small cell lung cancer (paraneoplastic syndrome)

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31
Q

myasthenia gravis - sex

A

MC seen in women

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32
Q

myasthenia gravis vs Lambert-Eaton - eyes involvement

A
  1. myasthenia gravis –> classically involves eyes

2. Lambert-Eaton –> eyes are usually spared

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33
Q

myasthenia gravis - symptoms

A
  1. ptosis
  2. diplopia
  3. weakness
    worsen with muscle use
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34
Q

Lambert-Eaton myasthenic syndrome - symptoms

A
  1. proximal muscle weakness
  2. autonomic symptoms (dry mouth, impotence)
    improves with muscle use
35
Q

autonomic symptoms of Lambert-Eaton myasthenic syndrome

A

dry mouth, impotence

36
Q

myasthenia gravis vs Lambert-Eaton - symptoms after muscle use

A
  1. myasthenia gravis –> worsen

2. Lambert-Eaton –> improves

37
Q

myasthenia gravis vs Lambert-Eaton - ACH inhibitor administration

A
  1. myasthenia gravis –> reversal of symptoms

2. Lambert-Eaton –> minimal effect

38
Q

fibromyalgia - most commonly seenin

A

females 20-50

39
Q

fibromyalgia - clinical manifestation

A

(1) chronic, widespread musculoskeletal pain associated with (2)stiffness, (3) paresthesias, (4) poor sleep, (5) fatigue , 6. cognitive disturbance (“fibro fog”)

40
Q

fibromyalgia - treatment

A
  1. regular exercise
  2. antidepressants (TCAs, SNRIs)
  3. anticonvulsants
41
Q

polymyalgia rhematica - treatment

A

rapid response to low dose corticosteroids

42
Q

polymyalgia rhematica - labs

A
  1. increased ESR
  2. increased CRP
  3. normal CK
43
Q

polymyalgia rhematica - sex and age

A

older women

44
Q

polymyalgia rhematica is associated with

A

temporal (giant cell) arteritis

45
Q

polymyalgia rhematica clinical manifestation

A
  1. pain and stiffness in shoulders and hips
  2. fever
  3. malaise
  4. weight loss
    (NO MUSCULAR WEAKNESS)
46
Q

polymyalgia rhematica - pain and stiffness IN

A

shoulders and hips

47
Q

polymyalgia rhematica - muscular weakness

A

NO MUSCULAR WEAKNESS

48
Q

polymyositis - clinical presentation

A

progressive SYMMETRIC proximal weakness

49
Q

polymyositis - most often involves (area of the body)

50
Q

polymyositis - histology

A

endomysial inflammation with CD8+ T cells

necrotic muscle fibers

51
Q

polymyositis - treatment

A

steroid followed by long term immunosuppressant therapy (methotrexate)

52
Q

dermatomyositis - clinical presentation

A
  1. progressive symmetric proximal weakness (like polyomyositis)
  2. malar rash (similar to SLE)
  3. heliotrope rash
  4. Gottron papules
  5. shawl (or V-) and face rash
  6. mechanics hands
53
Q

heliotrope rash

A

erythematous periorbital rash

54
Q

mechanics hands

A

Cracking (and hyperkeratosis) of the finger pad skin, commonly involving the first, second, and third fingers

55
Q

Gottron papules

A

red papules of the elbows, knees and knuckles (MCP, ICP)

56
Q

dermatomyositis - some cases are associated with

A

carcinoma (eg. gastric)

57
Q

dermatomyositis - histology

A

perimysial inflammation with perifascicular atrophy with CD4+ T cells

58
Q

dermatomyositis - treatment

A

steroid followed by long term immunosuppressant therapy (methotrexate) (same as polymyositis)

59
Q

dermatomyositis - labs

A
  1. increased CK
  2. ANA +
  3. anti-Jo-1 +
  4. anti-SRP +
  5. anti-Mi-2+
60
Q

polymyositis - labs

A

(same as dermatomyositis)

  1. increased CK
  2. ANA +
  3. anti-Jo-1 +
  4. anti-SRP +
  5. anti-Mi-2+
61
Q

polymyositis vs dermatomyositis - histology

A

dermatomyositis –>perimysial inflammation with perifascicular atrophy with CD4+ T cells
polymyositis –> endomysial inflammation with CD8+ T cells
necrotic muscle fibers

62
Q

polymyositis vs dermatomyositis according t ocells

A

polymyositis –> CD8

dermatomyositis –> CD4

63
Q

sarcoidosis is more common in (epidemiology)

A

african-american females

64
Q

sarcoidosis - characteristic (histology)

A

widespreed noncaseating granulomas

65
Q

origin of widespreed noncaseating granulomas in sarcoidosis

A

immune mediated

66
Q

sarcoidosis - CD4/CD8 ratio

A

elavated in BRONCHOALVEOLAR LAVAGE

67
Q

sarcoidosis - ‘‘paraneoplastic’’

A
  1. elevated serum ACE

2. hypercalcemia

68
Q

sarcoidosis - treatment

69
Q

sarcoidosis - presentation

A

asymptomatic except for enlarged lymph nodes

70
Q

sarcoidosis - findings on CXR

A

bilateral adenopathy and coarse reticular opacities

71
Q

sarcoidosis - chest CT

A

extensive hilar and mediastinal adenopathy

72
Q

sarcoidosis is associated with

A
  1. restrictive lung disease (interstitial fibrosis)
  2. erythema nodosum
  3. Lupus pernio (skin lesion on phase resembling Lupus)
  4. Bell palsy
  5. uveitis
  6. hypercalcemia
73
Q

sarcoidosis - pathophysiology of hypercalcemia

A

increased 1‐α hydroxylase activity of epithelioid histiocytes converts vitamin D to its active form

74
Q

sarcoidosis - histology of granulomas

A

epitheloid granulomas containing microscopic Schaumann and asteroid bodies (within Giant cells)

75
Q

sarcoidosis - clinical manifestation on the eye

76
Q

MC presenting symptom of sarcoidosis

A

dyspnea or cough

77
Q

localized scleroderma - labs

A

antibodies against to DNA topoisomerase II

78
Q

Raynaud phenomenon - mechanism

A

decreased blood flow to hte skin due to arteriolar (small vessel) vasospasm in response to cold or strell

79
Q

Raynaud phenomenon - presentation

A

color change from white (ischemia) to blue (hypoxia) to red (reperfusion)
MOST OFTEN IN THE FINGERS

80
Q

Raynaud phenomenon - most often in (area of the body)

A

fingers and toes

81
Q

Raynaud phenomenon - Reynuad disease vs syndrome

A

disease –> when 1ry (idiopathic)

syndrome –> 2ry to a disease process such as mixed connective tissue disease, SLE, CREST –> digital ulceration

82
Q

Reynuad disease vs syndrome according to digital ulceration

A

Reynuad syndrome

83
Q

Raynaud phenomenon - treatment

A

Ca2+ channel blockers

Musculoskeletal and Connective Tissue (19 decks)

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