Scleroderma (Systemic Sclerosis) Flashcards

(11 cards)

1
Q

What cytokines and inflammatory mediators are involved in constricture of microvasculature (rarefaction)?

A
  1. IL-6
  2. TFG-beta
  3. PDGF
  4. Endothelin
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2
Q

Role of IL-6 in pathogenesis

A
  1. ↑ TH-2 cytokine expression

2. High levels correlate with skin thickening

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3
Q

Role of IL-4, IL-13 (TH2 cytokines)

A
  1. Induce fibroblast proliferation

2. Induce synthesis of ECM

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4
Q

ANA pattern that is specific for SSc

A
  1. Anti-centromere pattern
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5
Q

Role of TGF-beta

A
  1. Master regulator of fibrosis; induces pro-fibrotic genes
  2. Secreted by macrophages normally
  3. SSc: secreted by myofibroblasts also
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6
Q

Role of CTGF (Connective Tissue Growth Factor)

A

Enhance fibrotic responses

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7
Q

Platelet derived growth factor (PDGF)

A
  1. From platelets, macrophages, endothelial cells, fibroblasts
  2. Attracts mesenchymal cells
  3. Induces transition of precursors to myofibroblasts phenotype
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8
Q

Renal crisis, though rare (5%), is associated with which antibody in Ssc?

A

Anti-RNA polymerase III

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9
Q

Manifestations of renal crisis

A
  1. Malignant HTN

2. Renal failure

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10
Q

Signs and symptoms of sSc-related pulmonary disease

A
  1. “Velcro Rales”

2. Progressive dyspnea

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11
Q

sSc antibody that is associated with high risk for development of interstitial lung disease

A

Anti-topoisomerase or Scl-70 antibody

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