[Five cases of systemic sclerosis with associated with intestinal pseudo-obstruction].

Abstract

Objectives: We experienced five cases of systemic sclerosis (SSc) associated with pseudo-obstruction, and evaluated their clinical characteristics and outcome.

Methods: Five patients with SSc were found to have intestinal pseudo-obstruction. We studied the clinical characteristics, treatments and outcome of these five case from medical records.

Results: The intestinal symptoms were free air in three and pneumatosis cystoides intestinalis (PCI) in two of the five patients. Cisapride was administered to all patients and octreotide and dinoprost to three patients, but the effects were not adequate and intravenous hyperalimentation (IVH) had to be introduced in four patients. Drug treatment was effective in patients in the early stage of intestinal symptoms, but IVH had to be introduced in patients with a long course. Septicemia occurred in two of the four patients on IVH and an infection occurred at the catheter insertion site in one patient. These two patients died of septicemia. Of five patients we experienced with SSc associated with intestinal pseudo-obstruction, two had PCI and four required IVH at home. Two of the four on home IVH developed septicemia.

Conclusions: Intestinal pseudo-obstruction is one of the poor prognostic factors for SSc.