Scleroderma

Incidence: 9:1,000,000 per y.

Prevalence: 300,000 Americans have scleroderma.

Male:female ratio is 1:4; highest in young African-American women.

More severe in Native Americans and African Americans.

10-y survival is 55–60%; presence of pulm Htn is a major prognostic predictor.

Severe hypotension secondary to hypovolemia

Hypoxia secondary to pulm Htn and restrictive disease

Failed intubation

GI reflux

Obliterative vasculopathy leading to pulm Htn

Restrictive lung disease

Renal crises

Intraop hypothermia-induced vasospasm

Scleroderma, or systemic sclerosis, is a chronic connective tissue disease generally classified as one of the autoimmune rheumatic diseases.

Targets skin, lungs, heart, GI system, kidneys, and MS system.

Onset generally occurs between 25–55 y.

Three features: Tissue fibrosis, vasculopathy of small blood vessels, autoimmune response.

Two major classifications: Limited and diffuse cutaneous scleroderma.

May have overlap syndromes with other rheumatic diseases.

Autoimmunity, genetics, hormones, and environmental factors may all play a role.

Autoantibodies: Antitopoisomerase in diffuse forms, anticentromere in limited form.

Twin studies suggest a limited genetic role.