connective tissue Flashcards

1
Q

what are the 5 cardinal signs of systemic sclerosis?

A
calcinosis
raynauds
esophageal dismotolity
sclerodactyly
telangiestasia
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2
Q

What diagnostic test indicate systemic sclerosis?

A

-anti-scl 70 positive in 70% of people with diffuse
(same as anti DNA topoisomerase)
-anticentromere antibody associated with 30% of limited form CREST syndrome
-ANA 95%
-RF in 25%
-elevated ESR

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3
Q

How does systemic sclerosis present?

A
puffy taut thick skin without wrinkles
thicken tendon sheath-loss ROM, crepitus
esophageal dysmotility
calcinosis
initially present as Raynauds
sclerodactyly
telangiestasia
malignant HTN-renal fail
pulmonary fibrosis
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4
Q

How do patients with systemic sclerosis die?

A

pulmonary fibrosis or renal fail

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5
Q

Who is most likely to get scleroderma or systemic sclerosis?

A

75% are women

50-60 yo

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6
Q

Where does the limited form of scleroderma present?

A

forearm, face, fingers, skin

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7
Q

what is the course of diffuse scleroderma?

A

rapid
widespread to skin
early involve of visceral organs(renal, GI, lungs, heart)

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8
Q

What does an x-ray of systemic sclerosis diffuse form show?

A

destructive changes in the joint
soft tisuse calcifications
periarticular resorption of bone

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9
Q

who is fibromyalgia most common in?

A

women 20-50years

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10
Q

what are the diagnostic requirements of Fibromyalgia?

A

muscle pain of at least 3 months duration
significant pain or tenderness in 11 of 19 tender points
normal neuro exam

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11
Q

What are some symptoms patients complain of in fibromyalgia?

A
  1. general muscle pain
  2. pain on exertion
  3. fatigue
  4. aching or burn or stiff (paresthesia)
  5. anxiety and depression
  6. stress aggrevates
  7. poor sleep
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12
Q

what is the treatment for fibromyalgia?

A
treat depression (SSRI, TCA)
cyclobenzaprine
NSAID
maintain good circulation
improve sleep quality
continue exercise
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13
Q

what type of treatment should be avoid in treating fibromyalgia?

A

glucocorticoids

opiates

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14
Q

Who does polymyalgia rheumitca present in?

A

most commonly women over 50

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15
Q

What is the clinical presentation of Polymyalgia rheumatic?

A

pain and stiffness in shoulders and hips and neck
weight loss
fever
fatigue
DOESNT cause muscle weakness
later stages can present with muscle atrophy

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16
Q

what condition is associated with PMR?

A

giant cell arteritis (temporal)

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17
Q

What are some diagnostic tests for PMR?

A
RF (-)
muscle biopsy (-)
normal CK
elevated ESR
can have normochromic monocytic anemia
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18
Q

Who does sarcoidosis most commonly present in?

A

blacks 20-40

women more than men

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19
Q

what is the etiology of sarcoidosis?

A

immune dysregulation

genetic background or possible environmental trigger

20
Q

What does the histology of sarcoidosis show?

A

noncaseating granulima of epitheliod macrophages
(process begins with acculumation of T cells and macros)
noncaseating granulomas contain Schaumann and asteriod bodies

21
Q

Where do noncaseating granulomas appear in sarcoidosis?

A

lung is most common

then skin and eye

22
Q

What is the treatment for PMR?

A

corticosteriods low dose

23
Q

What is the treatment for sarcoidosis?

A

steriods

70% recovery with minimal residual

24
Q

What is the clinical presenation of sarcoidosis?

A
  1. asymptomatic except for bilateral hilar adenopathy or reticular opacities
  2. hemoptysis
  3. cough, chest pain
  4. shortness of breath
25
Q

What is the onset for sarcoidosis?

A

insidious 40-70%

can be subacute a few weeks duration

26
Q

What are exam findings for sarcoidosis?

A

bilateral hilar adenopathy
splenomegaly
hepatomegaly
cutaneous lesions in eye (retinal opacities)

27
Q

what are some diagnostic tests for sarcoidosis?

A

CXR bilateral hilar adenopathy
elevated serum calcium (1 alpha hydroxylase mediated vitamin D activation by epitheliod macros)
elevated serum ACE levels
biopsy adenopathy

28
Q

What is the course of sarcoidosis?

A

progressive unrelenting or fluctuating

29
Q

What are some complications of sarcoidosis?

A
  1. bell’s palsy
  2. intersitital fibrosis(restrictive lung disease)
  3. erythema nodosum
  4. uvetitus
30
Q

what is systemic primary amyloidosis associated with?

A

multiple myeloma (anaplastic small cell cancer of bone marrow)

31
Q

What is systemic secondary amyloidosis?

A

AA- chronic disease of reactive nature

32
Q

Where are some places for local deposits of amyloid?

A
endocrine
atrial
amyloid angiopathy
cerebral -alziehmers
prion disease
33
Q

how does amyloidosis present?

A
  1. general ROS symptoms
    - weak
    - weight loss
    - dizzy
  2. later organ symptoms
34
Q

What is the presentation of SLE?

A
malar butterfly rash
photsensitivity
fever
pleuritic pain
joint pain
35
Q

who most commonly presents with SLE

A

black females under 20

36
Q

what is the presentation of SLE?

A
  1. acute, chronic or insidious onset
  2. joint pain
  3. fever
  4. buttery fly rash and photosensitivity, oral ulcers
  5. renal, cardiac and neuro symptoms
  6. can remit and relapse
  7. weight loss
37
Q

What is the etiology of SLE?

A
  1. fail self tolerance HLADR/HLADQ
  2. drug induced: d-peni, isonazid, hydrazine, procaninamide
  3. infectious agent (epstein barr titer positive) trigger hypersensitivity
38
Q

What organ systems are affected in SLE?

A

mucosal membranes (skin, lung, renal, cardiac, joint)

39
Q

what are some diagnostic tests for SLE?

A

anti-ds
Anti-smith
ANA
anti-phospholid (false negative with syphililis)

40
Q

How is SLE diagnosed?

A
  1. ANA, ds-DNA, Anti-sm, SSA and SSB antibodies
  2. 4 or more
    -malar rash
    -photosensivity
    -discoid rash
    -oral ulcers
    -serositis(pleuritis)(pericarditis)
    -renal (nephritis common cause of death)
    proteinuria
    -neuro (seizure or psychosis)
    -hemolytic anemia
    leukopenia
41
Q

how does erythema nodosum present?

A

can be with sarcoidosis or IBD

  1. tender poorly defined nodules
  2. nodules on extensor surface of lower extremity
  3. erythematous nodules
  4. edematous
42
Q

What is erythema nodosum associated with?

A

beta hemolytic strep infections
TB
drugs-sulfonamides, oral contraception
comorbid: sarcoidosis and IBD

43
Q

What is the most common cause of death in SLE?

A

renal fail-nephritis

44
Q

How is erythema nodosum diagnosed

A

biopsy the nodules

inflammed neutrophil infiltrate of subcutaneous fat and fascia

45
Q

What is the etiology of scleroderma?

A
systemic automimmune
B cell Th1 and T cell mediated
-T cell fibrogenesis of small vessels
-endothelial cell damage (renal, pulmonary, GI, skin, cardiac)
-vasocontrict and ischemia