Systemic Sclerosis (Scleroderma, SSc)

Difficult airway - Tight, fibrotic facial skin, microstomia (limited mouth opening), and a rigid neck make mask ventilation and laryngoscopy hard; oral and mucosal telangiectasias bleed easily. Plan a difficult airway with video laryngoscopy and awake fiberoptic in reserve.

Aspiration - Esophageal dysmotility and severe reflux are the rule; treat as a full stomach with aspiration prophylaxis and a secured airway.

Pulmonary hypertension and restrictive lung - Interstitial lung disease plus pulmonary arterial hypertension gives limited reserve; optimize oxygenation and ventilation and avoid raising pulmonary vascular resistance (hypoxia, hypercarbia, acidosis, hypothermia).

Vascular access and Raynaud - Vasospasm makes IV and arterial access difficult; keep the patient and room warm, warm fluids, and avoid situations that provoke digital ischemia.

Renal - Scleroderma renal crisis is a hypertensive emergency; avoid nephrotoxins, and note that ACE inhibitors are the treatment rather than a drug to stop.

Regional - Regional techniques can be helpful (sympatholysis aids perfusion) but tight skin and contractures make them technically harder; document neuropathy first.

An autoimmune connective-tissue disease combining widespread fibrosis with a small-vessel vasculopathy. Skin thickening and tightening (including of the face and mouth), Raynaud phenomenon, esophageal and gut dysmotility, interstitial lung disease with pulmonary arterial hypertension, myocardial fibrosis and conduction disease, and scleroderma renal crisis are the major organ effects. For the anesthesia provider almost every system that matters is involved: a fibrosed airway, severe reflux, stiff lungs with pulmonary hypertension, difficult vascular access from vasospasm, and fragile renal function.