Care of patients with scleroderma in the intensive care setting.

ABSTRACT

Scleroderma or systemic sclerosis (SSc) is a connective tissue disease (CTD) associated with fibrosing and vascular complications involving multiple organs. The care of these patients in the critical care setting is frequently challenging due to multiple complications and refractory organ involvement. However, awareness of specific organ involvement associated with scleroderma can allow many complications to be anticipated and effectively treated. Cardiac involvement can lead to arrhythmias and heart failure, whereas pulmonary involvement can be associated with pulmonary arterial hypertension, fibrosis, or both. Renal vascular disease and scleroderma renal crisis (SRC), once a uniformly fatal complication, is particularly important to recognize early, as it can be treated successfully. Gastrointestinal involvement can lead to bleeding, aspiration, obstruction, and malabsorption. Severe Raynaud may lead to digital ischemia and gangrene. Therapies must target involved organ system or organ systems. Corticosteroids, a mainstay for related CTDs, do not typically provide any benefit and may cause harm. Vasodilators can effectively treat vascular complications but must target the appropriate vascular bed. Proactive utilization of proton pump inhibitors, recognition of bleeding from gastrointestinal vascular ectasia, and nutritional support can considerably ameliorate gastrointestinal morbidities. Effective treatment of fibrotic complications remains elusive and is the current frontier for scleroderma therapeutics.