Elsevier

Critical Care Clinics

Volume 18, Issue 4, October 2002, Pages 819-839
Critical Care Clinics

Life-threatening complications of systemic sclerosis

https://doi.org/10.1016/S0749-0704(02)00023-4Get rights and content

Section snippets

Prevalence

Pulmonary arterial hypertension is common among patients with SSc, even in the absence of clinical signs and symptoms. In a sample of 34 consecutive patients with SSc who did not have a clinical diagnosis of pulmonary hypertension, pulmonary artery systolic pressure estimated from the velocity of the tricuspid regurgitation jet during Doppler echocardiography ranged from 15 to 95 mm Hg. Twelve of these patients (35%) had a pulmonary artery systolic pressure of 30 mm Hg or greater [5]. The

Renal crisis

Scleroderma renal crisis is associated with acute malignant hypertension that requires intensive care. In a large, retrospective, case-control study, a significant association between antecedent high-dose corticosteroid therapy and the development of scleroderma renal crisis was found [61]. As in hypertensive emergencies from other causes, patients may notice increased fatigue, malaise, dyspnea, headache, and blurred vision. Cardiac decompensation is often seen. The pathogenesis involves

Summary

Pulmonary arterial hypertension is common in patients with SSc. Fig. 1 shows the diagnostic and therapeutic approach to PAH in SSc. Doppler echocardiography may suggest the diagnosis, but RHC is necessary to confirm PAH and to measure vasoreactivity. Therapy is directed at the underlying connective tissue disease. Vasoreactive patients often benefit from therapy with high-dose calcium-channel blockers, but most patients are not vasoreactive. Intravenous epoprostenol and oral endothelin-1

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