Scleroderma (Systemic Sclerosis) Flashcards
(11 cards)
What cytokines and inflammatory mediators are involved in constricture of microvasculature (rarefaction)?
- IL-6
- TFG-beta
- PDGF
- Endothelin
Role of IL-6 in pathogenesis
- ↑ TH-2 cytokine expression
2. High levels correlate with skin thickening
Role of IL-4, IL-13 (TH2 cytokines)
- Induce fibroblast proliferation
2. Induce synthesis of ECM
ANA pattern that is specific for SSc
- Anti-centromere pattern
Role of TGF-beta
- Master regulator of fibrosis; induces pro-fibrotic genes
- Secreted by macrophages normally
- SSc: secreted by myofibroblasts also
Role of CTGF (Connective Tissue Growth Factor)
Enhance fibrotic responses
Platelet derived growth factor (PDGF)
- From platelets, macrophages, endothelial cells, fibroblasts
- Attracts mesenchymal cells
- Induces transition of precursors to myofibroblasts phenotype
Renal crisis, though rare (5%), is associated with which antibody in Ssc?
Anti-RNA polymerase III
Manifestations of renal crisis
- Malignant HTN
2. Renal failure
Signs and symptoms of sSc-related pulmonary disease
- “Velcro Rales”
2. Progressive dyspnea
sSc antibody that is associated with high risk for development of interstitial lung disease
Anti-topoisomerase or Scl-70 antibody