Introduction Patients with systemic sclerosis (SSc) may develop significant gastrointestinal involvement (GI) with associated nutritional impairment. When severe, this can progress to intestinal failure (IF) requiring home parenteral nutrition (HPN). Few outcome data are reported on these patients [1,2,3].

Methods Records were reviewed of all patients with SSc, who had been referred to a national IF centre and who required HPN between 1985 and 2012. Disease characteristics were evaluated and survival/outcome data compared to all patients requiring HPN in the IF centre.

Results 25 patients (5 male, median age: 55 (range 24–76)) with SSc received HPN. Median time from SSc diagnosis to HPN was 58 months (range 0–378). 24/25 patients had small intestinal involvement. 1 patient had severe colonic and pharyngeal dysmotility but could not tolerate enteral feeding. 17 patients had bacterial overgrowth. 7 reported pseudo-obstruction episodes and 5 had intestinal resections.

Prior to HPN, 7 patients had failed naso-enteric feeding. 10 had a gastrostomy or jejunostomy inserted; 7 of these patients received enteral feeding for < 1 yr. The remaining patients were commenced directly on HPN without enteral tube feeding because of the severity of dysmotility/associated co-morbidity. Only 2 patients were weaned off HPN (after 8 and 29 months) following successful medical optimisation. Survival on HPN is shown in table 1. No patients died from HPN-related complications. 17/18 died from underlying SSc disease. 1/18 died from malignancy. 7 patients survive, 6 remain on HPN (median duration: 50 months, range 27–173).

8 patients were trained to manage their central venous catheters and self-administer HPN. 17 patients relied on others to administer their HPN. Reported catheter complications in SSc patients (37,600 catheter days) included occlusion (26), sepsis (7), fracture (3) and site calcification (2). Catheter sepsis rate equated to 0.19 episodes per 1000 days (vs. 0.39 per 1000 days for all HPN patients at this centre). No patient with SSc developed IF-associated liver disease.

Conclusion This is the largest reported series of patients with SSc requiring HPN. Our data show that HPN offers a safe means of nutritional support for patients with severe SSc-related GI involvement, but that SSc-related mortality remains high. Notably, the SSc group had a lower catheter-related sepsis rate than all patients requiring HPN. Additionally, the majority relied on others for catheter care.

Disclosure of Interest None Declared

References

1. Ng et al. Arthritis Rheum. 1989;32:212–6,

2. Jawa et al. J. Rheum. 2012;39:1004–7,

3. Brown et al. Rheumatology. 2008;47:176–9