316. Retrospective Review of Patients with Systemic Sclerosis on Home Parenteral Nutrition

Abstract

Background: Patients with SSc may develop significant gastrointestinal (GI) involvement. When severe, this can lead to intestinal failure (IF) requiring home parenteral nutrition (HPN). However, few outcome data are reported for patients with SSc requiring HPN. Methods: Records were reviewed of all patients with SSc who commenced HPN, at a national IF centre, between 1990 and 2012. Disease characteristics and survival/outcome data were evaluated. Results: 25 patients [5 male, median age: 55 (range 24-76)] with SSc received HPN (37 200 catheter days). 24% had diffuse cutaneous SSc. The median time from SSc onset to HPN was 113 months (range 14- 389). All had small intestinal involvement. 80% had proven bacterial overgrowth. 44% had experienced at least one episode of pseudoobstruction. 16% had a small intestinal resection. Prior to HPN initiation, 6 patients failed a naso-enteric feeding trial. 10 patients had a gastrostomy or jejunostomy inserted; 7 of whom received enteral feeding for less than 1 year. The remaining 9 patients commenced HPN directly, without enteral tube feeding, because of the severity of their dysmotility/associated co-morbidity. Only 2 patients were weaned off HPN (after 8 and 29 months) following successful medical optimization. After 1 year, median BMI rose from 18.5 to 21.3. 3 patients received HPN for more than 10 years. The cumulative survivals on HPN after 1, 5 and 10 years were 75%, 37% and 23% respectively. No patients died from HPN-related complications. 16 died from causes related to their SSc. 1 died from malignancy. 8 patients survive, 7 of whom remain on HPN (median duration: 40 months, range 9-178). 9 patients were trained to manage their central venous catheters and self-administer HPN. 16 patients relied on others for their HPN administration. Reported catheter complications included non-thrombotic occlusion (0.70/1000 catheter days), sepsis (0.19/1000 catheter days) and central venous thrombosis (0.11/1000 catheter days). The sepsis rate for all HPN patients, at the same IF centre, is 0.39/1000 catheter days. No one developed IF-associated liver disease. Conclusion: This is the largest reported series of patients with SSc prescribed HPN, which is life-saving in patients with severe bowel involvement. Our study shows that HPN offers a safe means of nutritional support for patients with severe SSc-related GI involvement, but that SSc-related mortality remains high. The catheter-related sepsis rate of patients with SSc was low. In addition, the majority of patients with SSc relied on others for their catheter care.