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RMSK > 13 - Systemic Sclerosis > Flashcards

13 - Systemic Sclerosis Flashcards

1
Q

What is systemic sclerosis/scleroderma (SSc)?

A

Autoimmune or connective tissue disease characterized by thickening of the skin caused by accumulation of collagen, and by injuries to the smallest arteries

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2
Q

What is the prevalence of SSc in the US?

A

276 per million

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3
Q

What is the 10-year mortality rate of SSc?

A

23-45%

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4
Q

True or false: There is no demonstrated disease-modifying therapy for SSc

A

True

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5
Q

What are the subclassifications of SSc?

A
  • Limited cutaneous SSc (lcSSc)
  • Diffuse cutaneous SSc (dcSSc)
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6
Q

What is the pathophysiology of SSc?

A
  • Must incorporate the 3 cardinal features of the disease:
    • (1) vasculopathy (intimal hyperplasia, vessel obliteration, thrombosis)
    • (2) cellular and humoral autoimmunity (TGF-B, Th2 cytokines, PDGF, CTGF, chemokines)
    • (3) progressive visceral and vascular fibrosis in multiple organs (loss normal architecture, impaired function, organ failure)
  • Autoimmunity and altered vascular reactivity may be the earliest manifestations
  • Complex interplay between these processes is thought to initiate and then amplify the fibrotic process
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7
Q

Anti-PM-Scl AAb

  1. Main autoantigen target
  2. Frequency
  3. Clincal associations
A
  1. PM-Scl 100 7 PM-Scl 70 components of human exosome
  2. 5%
  3. Overlap w/ myositis
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8
Q

Anti-Th/To AAb

  1. Main autoantigen target
  2. Frequency
  3. Clincal associations
A
  1. Ribonuclease mitochondrial RNA processing & ribonuclease P complexes
  2. 5%
  3. lcSSc; ILD
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9
Q

Anti-U3RNP AAb

  1. Main autoantigen target
  2. Frequency
  3. Clincal associations
A
  1. 34-kDa basic protein fibrillarin
  2. 5%
  3. pHTN; skeletal muscle involvement; AfAm predominance
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10
Q

Anti-RNA polymerase III AAb

  1. Main autoantigen target
  2. Frequency
  3. Clincal associations
A
  1. RNA polymerase III
  2. 10%
  3. dcSSc; rapidly progressive skin dz; renal crisis
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11
Q

Anti-Scl-70 AAb

  1. Main autoantigen target
  2. Frequency
  3. Clincal associations
A
  1. Topoisomerase I
  2. 20%
  3. dcSSc; ILD
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12
Q

Anti-centromere AAb

  1. Main autoantigen target
  2. Frequency
  3. Clincal associations
A
  1. Centromere proteins
  2. 30%
  3. lcSSc; digital ischemia
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13
Q

What are the clinical manifestations of SSc?

A
  • Raynaud’s phenomenon
  • Digital ulcerations
  • Calcinosis
  • GI manifestations
  • Tendon friction rubs
  • Fibrosis of skin
  • Renal crisis
  • ILD
  • pHTN
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14
Q

What is Raynaud’s phenomenon?

A
  • Triphasic color change upon cold exposure
    • White –> blue –>
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RMSK (16 decks)

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