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
MC neuromascular junction disorder
Myasthenia gravis
26
myasthenia gravis vsLambert-Eaton according frequency
1. myasthenia gravis --> MC uromascular junction disorder | 2. Lambert-Eaton --> uncommon
27
pathophysiology of myasthenia gravis
autoantibodies to postsynaptic ACH receptor
28
pathophysiology of Lambert-Eaton myasthenic syndrome
autoantibodies to presynaptic Ca2+ channel --> decreased ACH release
29
myasthenia gravis is associated with
1. thymoma | 2. thymic hyperplasia
30
Lambert-Eaton myasthenic syndrome is associated with
small cell lung cancer (paraneoplastic syndrome)
31
myasthenia gravis - sex
MC seen in women
32
myasthenia gravis vs Lambert-Eaton - eyes involvement
1. myasthenia gravis --> classically involves eyes | 2. Lambert-Eaton --> eyes are usually spared
33
myasthenia gravis - symptoms
1. ptosis 2. diplopia 3. weakness worsen with muscle use
34
Lambert-Eaton myasthenic syndrome - symptoms
1. proximal muscle weakness 2. autonomic symptoms (dry mouth, impotence) improves with muscle use
35
autonomic symptoms of Lambert-Eaton myasthenic syndrome
dry mouth, impotence
36
myasthenia gravis vs Lambert-Eaton - symptoms after muscle use
1. myasthenia gravis --> worsen | 2. Lambert-Eaton --> improves
37
myasthenia gravis vs Lambert-Eaton - ACH inhibitor administration
1. myasthenia gravis --> reversal of symptoms | 2. Lambert-Eaton --> minimal effect
38
fibromyalgia - most commonly seenin
females 20-50
39
fibromyalgia - clinical manifestation
(1) chronic, widespread musculoskeletal pain associated with (2)stiffness, (3) paresthesias, (4) poor sleep, (5) fatigue , 6. cognitive disturbance ("fibro fog")
40
fibromyalgia - treatment
1. regular exercise 2. antidepressants (TCAs, SNRIs) 3. anticonvulsants
41
polymyalgia rhematica - treatment
rapid response to low dose corticosteroids
42
polymyalgia rhematica - labs
1. increased ESR 2. increased CRP 3. normal CK
43
polymyalgia rhematica - sex and age
older women
44
polymyalgia rhematica is associated with
temporal (giant cell) arteritis
45
polymyalgia rhematica clinical manifestation
1. pain and stiffness in shoulders and hips 2. fever 3. malaise 4. weight loss (NO MUSCULAR WEAKNESS)
46
polymyalgia rhematica - pain and stiffness IN
shoulders and hips
47
polymyalgia rhematica - muscular weakness
NO MUSCULAR WEAKNESS
48
polymyositis - clinical presentation
progressive SYMMETRIC proximal weakness
49
polymyositis - most often involves (area of the body)
shoulders
50
polymyositis - histology
endomysial inflammation with CD8+ T cells | necrotic muscle fibers
51
polymyositis - treatment
steroid followed by long term immunosuppressant therapy (methotrexate)
52
dermatomyositis - clinical presentation
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
heliotrope rash
erythematous periorbital rash
54
mechanics hands
Cracking (and hyperkeratosis) of the finger pad skin, commonly involving the first, second, and third fingers
55
Gottron papules
red papules of the elbows, knees and knuckles (MCP, ICP)
56
dermatomyositis - some cases are associated with
carcinoma (eg. gastric)
57
dermatomyositis - histology
perimysial inflammation with perifascicular atrophy with CD4+ T cells
58
dermatomyositis - treatment
steroid followed by long term immunosuppressant therapy (methotrexate) (same as polymyositis)
59
dermatomyositis - labs
1. increased CK 2. ANA + 3. anti-Jo-1 + 4. anti-SRP + 5. anti-Mi-2+
60
polymyositis - labs
(same as dermatomyositis) 1. increased CK 2. ANA + 3. anti-Jo-1 + 4. anti-SRP + 5. anti-Mi-2+
61
polymyositis vs dermatomyositis - histology
dermatomyositis -->perimysial inflammation with perifascicular atrophy with CD4+ T cells polymyositis --> endomysial inflammation with CD8+ T cells necrotic muscle fibers
62
polymyositis vs dermatomyositis according t ocells
polymyositis --> CD8 | dermatomyositis --> CD4
63
sarcoidosis is more common in (epidemiology)
african-american females
64
sarcoidosis - characteristic (histology)
widespreed noncaseating granulomas
65
origin of widespreed noncaseating granulomas in sarcoidosis
immune mediated
66
sarcoidosis - CD4/CD8 ratio
elavated in BRONCHOALVEOLAR LAVAGE
67
sarcoidosis - ''paraneoplastic''
1. elevated serum ACE | 2. hypercalcemia
68
sarcoidosis - treatment
steroids
69
sarcoidosis - presentation
asymptomatic except for enlarged lymph nodes
70
sarcoidosis - findings on CXR
bilateral adenopathy and coarse reticular opacities
71
sarcoidosis - chest CT
extensive hilar and mediastinal adenopathy
72
sarcoidosis is associated with
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
sarcoidosis - pathophysiology of hypercalcemia
increased 1‐α hydroxylase activity of epithelioid histiocytes converts vitamin D to its active form
74
sarcoidosis - histology of granulomas
epitheloid granulomas containing microscopic Schaumann and asteroid bodies (within Giant cells)
75
sarcoidosis - clinical manifestation on the eye
uveitis
76
MC presenting symptom of sarcoidosis
dyspnea or cough
77
localized scleroderma - labs
antibodies against to DNA topoisomerase II
78
Raynaud phenomenon - mechanism
decreased blood flow to hte skin due to arteriolar (small vessel) vasospasm in response to cold or strell
79
Raynaud phenomenon - presentation
color change from white (ischemia) to blue (hypoxia) to red (reperfusion) MOST OFTEN IN THE FINGERS
80
Raynaud phenomenon - most often in (area of the body)
fingers and toes
81
Raynaud phenomenon - Reynuad disease vs syndrome
disease --> when 1ry (idiopathic) | syndrome --> 2ry to a disease process such as mixed connective tissue disease, SLE, CREST --> digital ulceration
82
Reynuad disease vs syndrome according to digital ulceration
Reynuad syndrome
83
Raynaud phenomenon - treatment
Ca2+ channel blockers