Do CREST patients have pulmonary hypertension?
Status: Student (aka useless, know nothing)
Yes.
CREST (Calcinosis cutis, Raynaud, oEsophageal dysmotility, Sclerodactyly, Telangiectasia) is an old term used to describe a subset of patients with limited cutaneous scleroderma although it's recognised that these appear in diffuse cutaneous scleroderma too.
Limited cutaneous scleroderma vs. Diffuse cutaneous scleroderma are differentiated based on how much of the skin is involved. I guess that
may have implications on vascular access but you guys are all awesome.
GI problems are many (e.g. difficult mouth opening, reflux, GI bleeding, diarrhoea .......)
may have implications on intubation but again you guys are all awesome, so no problem there.
Lung problems are bad. Whichever form you have (limited or diffuse) you can get pulmonary arterial hypertension (+/- pulmonary fibrosis). Kidney problems are bad too but usually only in diffuse scleroderma. Cardiac conduction fibrosis is bad too. What can I say, it's not a good disease.
Source: Rheumatology cougar.
edit:
"Regional anesthesia may be technically difficult because of the skin and joint changes that accompany scleroderma. Attractive features of regional anesthesia include peripheral vasodilation and postoperative analgesia. Measures to minimize peripheral vasoconstriction include maintenance of the operating room temperature above 21C and administration of warmed intravenous fluids." - Stoelting's Anesthesia and Co-existing Disease (5th ed.)