CREST Syndrome

Pts with Hx of exposure to silica dust or PVC.

Usual age group is 30–50 y.

Fourfold to ninefold higher incidence in women than men; seen in all races.

In USA systemic sclerosis has an estimated incidence of 19 cases per million and prevalence of 240 cases per million (range: 138–286).

Pts more likely to have compromised renal function at baseline

Hypoxia from pulm Htn and/or restrictive lung disease

Difficult intubation from narrow mouth opening

Reflux and thereby aspiration, renal crises, restrictive lung disease, CHF, pulm Htn, difficult intubation due to small mouth opening, and keeping patient warm to avoid Raynaud phenomenon

C alcinosis, R aynaud phenomenon, E sophageal dysmotility, S clerodactyly, and T elangiectasia (CREST).

Symptoms involved in CREST or limited cutaneous systemic sclerosis are associated with the generalized form of systemic sclerosis.

Exact etiology of systemic sclerosis is unknown; the following pathogenic factors are always present: Endothelial cell injury, fibroblast activation, and cellular and humoral immunologic derangement.

Environmental factors, such as silica, industrial solvents, and radiation exposure, are all triggers or accelerators.

CMV, HHV5, and parvovirus are possible viral accelerators.