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MSS 2 > Connective Tissue Disease, Scleroderma and Polymyositis > Flashcards

Connective Tissue Disease, Scleroderma and Polymyositis Flashcards

1
Q

What are the connective tissue diseases?

A
  • RA
  • SLE
  • systemic sclerosis (scleroderma)
  • poly/dermatomyositis
  • Sjogren’s syndrome
  • overlap (MCTD)
  • undifferentiated CTD
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2
Q

What is Sjogren’s and how does it present?

A
  • autoimmune destruction of lacrimal and salivary glands.
  • presents as DRY EYES (keratoconjunctivitis sicca), DRY MOUTH (xerostomia), and recurrent dental caries in an older woman (aka SICCA syndrome).
  • “can’t chew a cracker, dirt in my eyes”
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3
Q

What antibodies will you see in Sjogren syndrome?

A
  • anti-SSA and anti-SSB
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4
Q

What should you think about when a pt presents with Raynaud’s phenomenon (CTD)?

A
  • systemic sclerosis (scleroderma)
  • SLE
  • RA
  • PM/DM (uncommon)
  • MCTD
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5
Q

What pts are prone for Raynaud’s?

A
  • those with positive ANA and abnormal capillary loops
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6
Q

What 3 diseases are associated with periungual erythema?

A
  1. poly/dermatomyositis
  2. SLE
  3. systemic sclerosis (scleroderma)
    * all 3 together is called mixed connective tissue disease (MCTD) or overlap disease.
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7
Q

In what 3 connective tissue diseases will you see bibasilar fibrosis; aka pulmonary fibrosis?

A
  1. poly/dermatomyositis= striated muscle
  2. scleroderma= smooth muscle
  3. MCTD
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8
Q

What is mononeuritis multiplex?

A
  • a painful, asymmetrical, asynchronous sensory and motor peripheral neuropathy involving isolated damage to at least 2 separate nerve areas.
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9
Q

** What are the 3 most common diseases that cause an asymmetric polyneuropathy (aka mononeuritis multiplex)? (TEST QUESTION)

A
  • SLE, RA, or PAN (vasculitis)
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10
Q

Does myositis define polymyositis?

A

YES

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11
Q

What antibody goes with MCTD?

A
  • anti-RNP
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12
Q

*** What are the characteristic signs of poly/dermatomyositis?

A
  • proximal muscle weakness (girdle and hips).
  • muscle enzyme elevation (creatine kinase and aldolase)
  • gottrons sign (rash on hands)
  • shawl’s sign
  • calcinosis cutis
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13
Q

At what age does poly/dermatomyositis occur?

A
  • 5-14
  • 45-54
  • females more
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14
Q

What antibodies are associated with Poly-Dermatomyositis?

A
  • anti-Jo-1
  • anti-SRP
  • ANA
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15
Q

Will you see an abnormal EMG with poly-dermatomyositis?

A
  • YES

* do unilateral EMG

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16
Q

How do we manage poly-dermatomyositis?

A
  • steroids
  • cytotoxics
  • methotrexate
  • azathioprine
  • IV immunoglobulin if severe
17
Q

What is systemic sclerosis (scleroderma)?

A
  • autoimmune disorder characterized by sclerosis of skin and visceral organs.
  • classically presents in middle-aged females (30-50 years).
18
Q

What is characteristic of systemic sclerosis (scleroderma)?

A
  • thickening of the skin proximal to the MCP joints.
19
Q

What is the pathogenesis of scerloderma?

A
  • fibroblast activation leads to deposition of collagen and FIBROSIS of the skin.
  • endothelial dysfunction leads to inflammation, vasoconstriction, and secretion of growth factors.
  • technically a MICROANGIOPATHY of the capillaries.
20
Q

What is the prototype of scleroderma?

A
  • CREST syndrome= Calcinosis/anti-cintromere antibodies, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly and Telangiectasias of the skin
21
Q

What is the sclerodactyly?

A
  • distal thickening
22
Q

What is the life-threatening complication that occurs in 10% of pts with scleroderma?

A
  • pulmonary fibrosis
23
Q

What antibody is seen in 80% of pts with scleroderma?

A
  • anti-centromere antibody (ACA)
24
Q

What is it called when pts with scleroderma lose the distal tufts of their digits?

A
  • acroosteolysis from erosion
25
Q

How does distal differ from diffuse scleroderma?

A
  • limited= skin changes distal to elbow/wrist. Telangiectasias are often involved and dilated capillaries loops.
    diffuse= skin changes spread proximal to elbow with drunk tendons involved. Associated with renal, cardiac, GI, and pulmonary fibrosis
26
Q

How do we treat pulmonary fibrosis in pts with scleroderma?

A
  • endothelin-1 receptor antagonists
  • phosphodiesterase type 5 inhibitors (sildenafil)
  • prostanoids
27
Q

How do we manage scleroderma?

A
  • NSAIDs for joint pain.
  • Penicillamine for skin and GI.
  • Nifedipine for Raynauds.
  • ACE inhibitors for HTN
  • Methotrexate, 5-FU…

MSS 2 (15 decks)

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