Outline the clinical manifestations of the CREST syndrome, and how these might influence the management of such a patient in Intensive Care.
CREST syndrome refers to the predominantly cutaneous rheumatological condition with Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly and Telangiectasia. Associated conditions include scleroderma (with additional arthralgias, myalgias, contractures, pulmonary fibrosis and pulmonary hypertension, renal impairment etc.). Patients with CREST syndrome therefore may have many manifestation that may complicate ICU management. Some of the many resultant potential problems include difficult intubation (limited mouth opening), risk of aspiration (and oesophageal perforation with TOE), malabsorption and nutritional deficiencies, limited respiratory reserve, risk of digital ischaemia with radial arterial lines and vasoconstrictors, skin breakdown/pressure area, and propensity to renal failure.
CREST stands for:
It is a member of the scleroderma family of disorders. In fact, its "scleroderma light" - the limited form of systemic sclerosis.
The factors which influence the ICU management of scleroderma therefore also apply to CREST. These are discussed in greater detail in the answer to Question 28 from the first paper of 2012. That answer is reproduced below:
Farber, Harrison W., Robert W. Simms, and Robert Lafyatis. "Analytic Review: Care of Patients With Scleroderma in the Intensive Care Setting." Journal of intensive care medicine 25.5 (2010): 247-258.
Legerton 3rd, C. W., Edwin A. Smith, and Richard M. Silver. "Systemic sclerosis (scleroderma). Clinical management of its major complications."Rheumatic diseases clinics of North America 21.1 (1995): 203-216.