Scleroderma

• Progressive systemic sclerosis:Inflammation, Vascular sclerosis, Fibrosis of skin and

viscera
• Tissue edema
• Some Pts have CREST syndrome, C: calcinosis, R: Raynaud’s phenomenon, E:
esophageal hypomotality, S: scleroderma, T: Telengectesia
• F>M, Age 20-40
• Pregnancy ↑ the progression of the disease in 50% of the Pt.

A: flexion contracture→ difficult airway due to limited mouth opening, ↓ motility, ↓ LOS
tone→ GERD→ need RSI
B: A major cause of M&M, Chronic aspiration, Diffuse lung fibrosis→ restrictive lung
disease, ↓ diffusion capacity
C: risk of IHD, fibrosis of the conductive system→ arrhythmia, cardiomyopathy→ CHF,
systemic and pulmonary HTN, pericarditis→ effusion → tamponade, with systemic HTN→ ↓
intravascular volume→ hypotension , Raynaud’s phenomenon in 70%→ risk of ischemia
with art-line
CNS: peripheral and cranial nerve neuropathy
o Eye: conjunctivitis and corneal abrasion
Renal: Renal artery obstruction→ accelerated HTN and RF→ the most common cause of
death
GI: bacterial over growth due to hypomotility→ malabsorption syndrome → ↓ vit K
dependent factors→ coagulation abnormality
Drugs: immunosuppressant , skeletal muscle myopathy→ ↑ sensitivity to muscle relaxant
Others:
 Regional anesthesia is
• difficult to do due to thick skin
• good for them→ sympathectomy→vasodilation
• may be prolonged due to vasoconstriction→ ↓ absorption
 avoid hypothermia→ ↑ vasoconstriction
 they are sensitive to opioids.
thick skin with edema→ difficult IV access