Scleroderma (Systemic Sclerosis)

Definition:

A chronic connective tissue disorder leading to widespread fibrosis

 

Pathophysiology:

Immune damage to endothelial cells -> ROS, cytokines -> fibroblasts -> increased collagen deposition (of normal composition) + skin thickening

 

Presentation:

  • Raynaud phenomenon
  • Skin: [degree of involvement predicts prognosis]: cutaneous fibrosis -> sclerodactyly (claw-like appearance of the hands), calcinosis cutis (subcutaneous calcification), “mouse-like” face and “purse-string” mouth
  • GI: dysphagia\reflux from esophageal immobility (90%), bowel dilatation
  • Pulmn: [MCC of death in scleroderma]: interstitial fibrosis, pulmn HTN
  • Scleroderma renal crisis: malignant HTN, ARF
  • Cardiac: myocardial fibrosis and pericarditis
  • Endocrine: amenorrhea, infertility, hypothyroidism

 

scleroderma 4

Diagnosis:

  • ↑ ANA (sensitive, not specific)
  • Anti-centromere: very specific for the limited form
  • Anti-topoisomerase I: very specific for the diffuse form

 

Treatment:

No cure, symptomatic treatment, organ-based approach:

  • D-penicillamine for skin
  • Ca channel blocker for Raynaud
  • ACE for renal HTN

 


Download the PDF version: here


References:

  • Step up to medicine
  • Pocket medicine
  • Toronto notes

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