rheumatoid diseases (except RA and gout) Flashcards

1
Q

systemic juvenile idiopathic osteoarthritis: what is this?

A

systemic arthritis in <16 y/o

presents with
1) arthritis (+2 joints)
2) salmon pink macular rash
3) daily spiking fevers

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2
Q

systemic juvenile idiopathic osteoarthritis: in who?

A

children <16 y/o

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3
Q

systemic juvenile idiopathic osteoarthritis: presentation?

A

arthritis commonly in +2 joints
daily spiking fevers
salmon pink macular rash

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4
Q

arthritis in +2 joints, pink macular rash, fever in 12 y/o. what is this suggestive of?

A

systemic juvenile idiopathic arthritis

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5
Q

what is systemic juvenile idiopathic arthritis associated with?

A

anterior uveitis

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6
Q

systemic juvenile idiopathic arthritis: lab?

A

leukocytosis, thrombocytosis, anemia
elevated ESR and CRP

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7
Q

systemic juvenile idiopathic arthritis: ESR and CRP?

A

elevated

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8
Q

systemic juvenile idiopathic arthritis: treatment?

A

NSAIDs
steroids
methotrexate
TNF inhibitors

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9
Q

Sjogren: what is this?

A

auto-immune disorder characterized by destruction of exocrine glands by lymphocytic infiltrates

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10
Q

Sjogren: what gets destructed?

A

exocrine glands, especially lacrimal and salivary

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11
Q

which glands gets most commonly destructed in Sjogren?

A

lacrimal and salivary

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12
Q

which cell type infiltrate glands in Sjogren?

A

lymphocytes

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13
Q

Sjogren: who gets mostly affected?

A

females 40-60 y/o

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14
Q

Sjogren: symptoms?

A
  • inflammatory joint pain
  • dry, sandy feeling eyes (keratoconjunctivitis sicca)
  • dry mouth (xerostomia)
  • bilateral parotid enlargement
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15
Q

what causes the dry eyes (keratoconjunctivitis sicca) in Sjogren?

A

decreased tear production and subsequent corneal damage

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16
Q

Other word for dry eyes in Sjogren?

A

keratoconjunctivitis sicca

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17
Q

Other word for dry mouth in Sjogren?

A

Xerostomia

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18
Q

joint pain with dry eyes/mouth and enlarged parotides. what can it be?

A

sjogren

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19
Q

sjogren: what is the cause of xerostomia?

A

decreased saliva production -> mucosal damage

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20
Q

what can you see on a tongue in sjogren?

A

fissuring

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21
Q

sjogren: which antibodies?

A

ANA +
RF + (can be positive without RA)
SS-A and SS-B

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22
Q

what are antiribonucleoprotein antibodies?

A

SS-A and SS-B

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23
Q

what kind of antibodies are SS-A and SS-B?

A

antiribonucleoprotein

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24
Q

in which diseases may SS-A and SS-B be seen?

A

sjogren and SLE

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25
Q

is sjogren common as a primary disorder?

A

yes

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26
Q

what can sjogren be secondary to?

A

RA, SLE, SS (systemic sclerosis)

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27
Q

sjogren: complications?

A

dental caries
MALT lymphoma
risk of giving birth of baby with neonatal lupus

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28
Q

MALT lymphoma: complication of what?

A

sjogren

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29
Q

dental caries: complication of what?

A

sjogren

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30
Q

sjogren: risk of having baby with what kind of disorder?

A

neonatal lupus

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31
Q

sjogren: how to confirm diagnosis?

A

labial salivary gland biopsy

(focal lymphocytic sialadenitis)

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32
Q

what do you see on the labial salivary gland biopsy in sjogren?

A

focial lymphocytic sialadenitis

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33
Q

other word for inflammation of a salivary gland?

A

sialadenitis

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34
Q

septic arthritis: common causes? (pathogens)

A

s. aureus
streptococcus
neisseria gonorrea

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35
Q

septic arthritis: mostly bilateral or unilateral?

A

unilateral

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36
Q

septic arthritis: presentation?

A

swollen, red, painful joint
synovial fluid purulent

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37
Q

septic arthritis: synovial fluid?

A

purulent (WBC >50,000/mm3)

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38
Q

septic arthritis: treatment?

A

antibiotics, aspiration (needle), drainage (cut) to prevent irreversible joint damage

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39
Q

what do you do in septic arthritis to prevent irreversible joint damage?

A

drainage (+/- debridement)

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40
Q

difference joint drainage and aspiration?

A

aspiration: needle to let fluids out

drainage: cut it open en let fluids come out

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41
Q

disseminated gonococcal infection: how does it get transmitted? how does it present?

A

STI that presents as either a
1) purulent arthritis (e.g. knee)
2) or triad from polyarthralgia, tenosynovitis (hand), dermatitis (pustules)

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42
Q

what is the triad of disseminated gonoccal infection?

(presents as this triad or as a purulent arthritis)

A

polyarthralgia
tenosynovitis (e.g. hand)
dermatitis (pustules)

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43
Q

Pain at multiple joints, pustules and a tenosynovitis of the hand. What is this suggestive of?

A

disseminated gonococcal infection

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44
Q

what are the types of seronegative spondyloarthritis?

A

psoriatic arthritis
ankylosing spondylitis
IBD
reactive arthritis

(PAIR)

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45
Q

psoriatic arthritis
ankylosing spondylitis
IBD
reactive arthritis

what are these?

A

seronegative spondyloarthritis

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46
Q

what characterizes seronegative spondyloarthritis?

A

no RF
association with HLA-B27

shared symptoms:
- inflammatory back pain
- peripheral arthritis,
- enthesitis
- dactylitis
- uveitis

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47
Q

HLA-B27 associated with what?

A

seronegative spondyloarthritis (bechterew, IBD, reactive, psoriatic arthritis)

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48
Q
  • inflammatory back pain
  • peripheral arthritis,
  • enthesitis
  • dactylitis
  • uveitis

shared symptoms of what?

A

seronegative spondylarthritis

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49
Q

which type of arthritis are NEGATIVE for RF?

A

seronegative spondyloarthritis
(bechterew, reactive, psoriatic arthritis, IBD)

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50
Q

what is dactylitis?

A

swelling of fingers, ‘sausage’ fingers

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51
Q

what is enthesis?

A

inflamed insertion sites of tendons, e.g. Achilles

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52
Q

characteristics of back pain in seronegative spondylarthritis?

A

worse in morning, better with movement

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53
Q

Psoriatric arthitis: arthritis in combination with what?

A

skin psorias and nail lesions

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54
Q

psoriatric arthritis: involvement of joints symmetric or asymmetric?

A

asymmetric

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55
Q

psoriatric arthritis: xray?

A

dactylitis and pencil-in-a-cup deformity

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56
Q

pencil-in-a-cup deformity of DIP on xray. suggestive of what?

A

psoriatric arthritis

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57
Q

how often is psoriatric arthritis seen in psoriatric patients?

A

fewer than 1/3

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58
Q

ankylosing spondylitis: other name?

A

Bechterew

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59
Q

Bechterew: other name?

A

ankylosing spondylitis

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60
Q

ankylosing spondylitis: involvement of which joints?

A

spine and sacroiliac joints

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61
Q

ankylosing spondylitis: symmetric or asymmetric?

A

symmetric

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62
Q

complications ankylosing spondylitis?

A

joint fusion (ankylosis), uveitis, aortic regurgitation
restrictive lung disease

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63
Q

symmetric inflammation of sacroiliac joints. what kan it be?

A

ankylosing spondylitis (Bechterew)

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64
Q

ankylosing spondylitis: what happens to he spine?

A

vertebral fusion -> bamboo spine

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65
Q

ankylosing spondylitis: what can happen if costovertebral and costosternal ankylosis occurs?

A

restrictive lung disease

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66
Q

what can lead to restrictive lung disease in ankylosing spondylitis?

A

ankylosis of costovertebral and costosternal joints

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67
Q

how to assess disease severity of restrictive lung disease in ankylosing spondylitis?

A

degree of chest expansion

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68
Q

ankylosing spondylitis: more common in who?

A

males (20-40 y/o)

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69
Q

ankylosing spondylitis: age of onset?

A

20-40

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70
Q

which GI diseases are associated with seronegative spondylarthritis?

A

crohn and ulcerative colitis (IBD)

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71
Q

reactive arthritis: triad?

A

conjunctivitis
urethritis
arthritis

(can’t see, can’t pee, can’t bend my knee)

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72
Q

can’t see, can’t pee, can’t bend my knee

what is this?

A

reactive arthritis

(conjunctivitis, urethritis, arthritis)

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73
Q

reactive arthritis is what kind of arthritis?

A

seronegative spondyloarthritis
(RF negative, HLA-B27 associated)

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74
Q

psoriatic arthritis is what kind of arthritis?

A

seronegative spondyloarthritis
(RF negative, HLA-B27 associated)

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75
Q

bechterew is what kind of arthritis?

A

seronegative spondyloarthritis
(RF negative, HLA-B27 associated)

76
Q

mnemonic reactive arthritis symptoms?

A

can’t see, can’t pee, can’t bend my knee

77
Q

menmonic reactive arthritis bacteria?

A

She Caught Every Student Cheating Yesterday (and overREACted)

Shigella Campylobacter E.coli Salmonella Chlamydia Yersinia

78
Q

which pathogens cause reactive arthritis?

A

Shigella Campylobacter E.coli Salmonella Chlamydia Yersinia

(she caught every student cheating yesterday and overreacted)

79
Q

SLE: abbreviation for what?

A

systemic lupus erythematosus

80
Q

SLE: what is it?

A

relapsing-remitting auto-immune disease

classic presentation: rash, joint pain, fever in female of reproductive age

81
Q

SLE: which type of hypersensitivity reactions are involved?

A

type III: immune complex (=> most common)

type II: IgE, IgM

82
Q

How fast is a type III hypersensivity reaction?

A

1-3 weeks

83
Q

How fast is a type II hypersensitivity reaction?

A

hours to days

84
Q

what is a type III hypersensitivity reaction?

A

immune complex
1-3 wks

85
Q

what is a type II hypersensitivity reaction?

A

IgG, IgM
hours-days

86
Q

which type of hypersensitivity reaction causes most organ damage in SLE?

A

type III immune complex
1-3 weeks

87
Q

SLE is associated with a deficiency of what kind of proteins?

A

complement
(e.g. C1q, C4, C2)

-> reduced clearance of immune complexes

88
Q

SLE is associated with a reduced clearance of what?

A

immune complexes

(due to complement deficiency)

89
Q

what is an immune complex?

A

combination of an antibody with its antigen (can be multiple)

must be cleared, if not ->auto immune

90
Q

rash, joint pain, fever in female of reproductive age

suggestive of?

A

SLE

90
Q

classic presentation of SLE?

A

fever, rash and joint pain in female of reproductive age

91
Q

increased prevalence of SLE in which populations in the US?

A

black, hispanic, asian, caribbean

92
Q

heart condition associated with SLE?

A

LSE (Libman Sacks Endocarditis)

sterile thrombotic vegetations

93
Q

what is endocarditis?

A

inflammation of the inner lining of the heart’s chambers and valves

94
Q

which important complications can be caused by SLE?

A

LSE and lupus nephritis

95
Q

what is lupus nephritis?

A

glomerular deposition of DNA-anti-DNA immune complexes

96
Q

which immune complexes are deposited in lupus nephritis?

A

DNA-anti-DNA

97
Q

what is glomerular deposition of DNA-anti-DNA immune complexes?

A

lupus nephritis

98
Q

symptoms of lupus nephritis?

A

hematuria and proteinuria

99
Q

causes of death in SLE?

A

renal, infection and cardiovascular (accelerated CAD)

100
Q

what is the most common cause of death in SLE?

A

renal disease

101
Q

mnemonic causes of death in SLE?

A

patients die with Redness In their Cheeks

renal - infection - cardiovascular

102
Q

SLE with anti-SSA: increased risk of giving birth to baby with what?

A

neonatal lupus

103
Q

symptoms neonatal lupus?

A

neonatal heart block
periorbital/diffuse rash
transaminitis
cytopenia

(present at birth)

104
Q

what kind of antibody in a person with SLE gives increased risk of neonatal lupus?

A

anti-SSA

105
Q

mnemonic SLE symptoms?

A

RASH OR PAIN

Rash
Arthritis
Serositis
Hematological (cytopenia)

Oral/nasopharyngeal ulcers
Renal disease

Photosensitivity
ANA
Immunological
Neurological symptoms

106
Q

is arthritis in SLE erosive?

A

no

107
Q

what kind of serositis in SLE?

A

pleuritis, pericarditis

108
Q

what kind of hematological symptoms in SLE?

A

cytopenias

109
Q

what kind of rash in SLE?

A

malar or discoid

110
Q

what kind of ulcers in SLE?

A

oral and nasopharyngeal ulcers, painless

111
Q

what is ANA short for?

A

antinuclear antibodies

112
Q

neurological symptoms in SLE?

A

seizures, psychosis

113
Q

antibodies in SLE?

A

anti-nuclear antibody
anti-dsDNA
anti-Smith
antiphospholipid

ANA, anti-dsDNA, anti-Sm, antiphospholipid

114
Q

which antibody is specific for SLE?

A

anti-Smith (anti-Sm)

115
Q

is positive ANA always associated with an auto-immune disease?

A

no

116
Q

mixed connective tissue disease: which antibody?

A

speckled ANA (=anti-U1 RNP)

117
Q

mixed connective tissue disease: features of which diseases?

A

SLE, SS, polymyositis

118
Q

antiphospholipid syndrome: if secondary, to what?

A

SLE

119
Q

can antiphospholipid syndrom be a primary disease?

A

yes

120
Q

antiphospholipid syndrome: diagnosis based on what?

A

clinical criteria:
1) history of thrombosis (arterial or venous)
OR
2) spontaneous abortion

with lab findings of antiphospholipid antibodies

121
Q

which are the antiphospholipid proteins?

A

lupus anticoagulant
anti B2 glycoprotein I
anticardiolipin

122
Q

anticardiolipin is what kind of protein?

A

antiphospholipid

123
Q

anti b2 glycoprotein is what kind of protein?

A

antiphospholipid

124
Q

lupus anticoagulant is what kind of protein?

A

antiphospholipid

125
Q

antiphospholipid syndrom: treatment?

A

systemic anticoagulation

126
Q

history of thrombosis and lupus anticoagulant positive

suggestive of?

A

antiphospholipd syndrome

127
Q

history of spontaneous abortion and anticardiolipin positive

suggestive of?

A

antiphospholipid synrome

128
Q

what test can anticardiolipin influence?

A

syphilis test (VDRL/RPR)
-> false positive

129
Q

which test can lupus anticoagulant influence?

A

can cause prolonged PTT

not correct by addition of normal platelet free plasma

130
Q

polymyalgia rheumatica: symptoms?

A

pain and stiffness in proximal muscles (shoulders, hip)

often with systemic symptoms (fever, malaise, weight loss)

131
Q

does polymyalgia rheumatica cause muscle weakness?

A

no

132
Q

what is polymyalgia rheumatica associated with?

A

giant cell (temporal) arteritis

133
Q

polymyalgia rheumatica: most common in who?

A

females >50 y/o

134
Q

woman 55 y/o, stiff and painful shoulders and hips. no weakness

what is this suggestive of?

A

PMR

135
Q

polymyalgia rheumatica: lab values?

A

elevated ESR and CRP
normal CK

136
Q

polymyalgia rheumatica: creatinine kinase elevated?

A

no

137
Q

polymyalgia rheumatica: treatment?

A

low dose glucocorticosteroids

138
Q

polymyalgia rheumatica: how fast is the response to low dose corticosteroids?

A

fast

139
Q

fibromyalgia: most common in who?

A

females 20-50 y/o

140
Q

fibromyalgia: symptoms?

A

chronic, widespread musculoskeletal pain

associated with ‘tender points, stiffness, paresthesias, poor sleep, fatigue, cognitive disturbances ‘fibro fog’

141
Q

which rheumatic disease is associated with ‘tender points’?

A

fibromyalgia

142
Q

45 y/o woman with diffuse chronic pain in joints with tender points, poor sleep and fatigue

suggestive of?

A

fibromyalgie

143
Q

fibromyalgie: how are the inflammatory markers (ESR, CRP)?

A

normal

144
Q

Fibromyalgia: treatment?

A

exercise, antidepressants (TCA), neuropathic pain agents (gabapentin)

145
Q

what is gabapentin?

A

neuropathic pain agent

used in fibromyalgia

146
Q

what is a neuropathic pain agent used in fibromyalgia?

A

gabapentin

147
Q

Polymyositis/dermatomyositis: nonspecific antibodies/lab values

A

ANA +
elevated CK

148
Q

polymyositis/dermatomyositis: specific antibodies

A

anti-Jo-1 (histidyl-tRNA synthetase)
anti-SRP (signal recognition particle)
anti-Mi-2 (helicase)

149
Q

anti-Jo-1: antibody against?

A

histidyl-tRNA synthetase

150
Q

anti-SRP: antibody against?

A

signal recognition particle

151
Q

anti-Mi-2: antibody against?

A

helicase

152
Q

anti-Jo-1: specific for which diseases?

A

polymyositis/dermatomyositis

153
Q

anti-SRP: specific for what?

A

polymyositis/dermatomyositis

154
Q

anti-Mi-2: specific for what?

A

polymyositis/dermatomyositis

155
Q

Polymyositis: symptoms?

A

progressive symmetric proximal muscle weakness

156
Q

polymyositis: symmetric or asymmetric?

A

symmetric

157
Q

polymyositis: which muscles most often?

A

proximal muscles, most often shoulders

158
Q

polymyositis: underlying pathology?

A

endomysial inflammation with CD8+ cells

endomysium: surrounds each individual muscle fiber/cell

159
Q

what is the endomysium?

A

the sheath surrounding each individual muscle fiber

160
Q

polymyositis: weakness?

A

yes

161
Q

person with progressive symmetrical weakness in shoulders

no skin involvement

suggestive of?

A

polymyositis

162
Q

dermatomyositis: symptoms?

A

similar to polymyositis (progressive symmetric proximal muscle weakness) with skin involvement:
- Gottron papules
- facial erythema
- shawl and face rash
- mechanic’s hands

163
Q

dermatomyositis: dermatological symptoms?

A
  • Gottron papules
  • facial erythema (eyelids)
  • face and shawl rash
  • mechanic’s hands
164
Q

what are gottron papules?

A

red, dry/flaky, bumps overlying the knuckles of the fingers

pathognomic for dermatomyositis

165
Q

how does the facial erythema in dermatomyositis commonly look?

A

heliotrope (violet) edema of the eyelids

face and shawl edema

166
Q

what color is heliotrope edema?

A

violet

heliotrope edema of the eyelids often seen in dermatomyositis

167
Q

what are ‘mechanic’s hands’?

A

thickening, cracked, irregular, ‘dirty’ skin of fingers due to hyperkeratosis

often seen in dermatomyositis

168
Q

dermatomyositis: pathology?

A

perimysial inflammation and atrophy with CD4+ cell involvement

perimysium: sheet of connective tissue surrounding a bundle of muscle fibers

169
Q

what cells are involved in polymyositis?

A

CD8+

170
Q

what cells are involved in dermatomyositis?

A

CD4+

171
Q

which sheet in a muscle is involved in polymyositis?

A

endomysium

(surrounds individual muscle cell)

172
Q

which sheet in a muscle is involved in dermatomyositis?

A

perimysium

(surrounds bundle of muscle cells)

173
Q

dermatomyositis gives increased risk of what other disease?

A

occult malignancy

(can’t find the primary tumor)

173
Q

person with progressive, symmetric weakness of the shoulders and red bumps on joints of the fingers

suggestive of?

A

dermatomyositis

174
Q

myositis ossificans: what is this?

A

heterotopic ossification in skeletal muscle (e.g. quadriceps)

175
Q

heterotopic: meaning?

A

in the wrong place

176
Q

heterotopic ossification: what is this?

A

extraskeletal bone in muscle and soft tissue

177
Q

myositis ossificans: associated with what?

A

blunt muscle trauma

178
Q

myositis ossificans: presentation?

A

painful soft tissue mass

179
Q

myositis ossificans: imaging?

A

eggshell calcification

180
Q

myositis ossificans: histology?

A

metaplastic bone surrounding area of fibroplastic proliferation

181
Q

myositis ossificans: benign or malignant?

A

benign

182
Q

what may myositis ossificans be mistaken for?

A

osteosarcoma

(myositis ossificans is benign)

183
Q

person develops hard, painful mass in quadriceps after blunt trauma

imaging: eggshell calcification near femur

what benign condition can this be?

A

myositis ossificans

184
Q

RA: most commonly affected joints?

A

hands, wrists, and knees

185
Q

osteoarthritis: most commonly affected joints?

A

hands, hips, and knees