Home parenteral nutrition--an effective and safe long-term therapy for systemic sclerosis-related intestinal failure

To examine the outcome in patients with SSc requiring parenteral nutrition (PN), and to compare their clinical characteristics with those of other SSc patients and of patients requiring PN/home parenteral nutrition (HPN) for other conditions.

Retrospective review of SSc and Intestinal Failure Unit databases at a tertiary referral centre for SSc/national unit for intestinal failure over a 13-yr period.

Eight patients with SSc requiring PN during the study period were identified (2 males, 6 females: median age at commencement of PN 51 yrs, range 42-56 yrs). All patients commencing PN had bacterial overgrowth and malabsorption not responding to antibiotic therapy. The median duration of PN therapy in the eight patients was 40 months (range 0.8-192 months). Between them the eight patients had a total of 13,851 catheter-use days and only two line infections (0.14/1000 catheter days), a lower rate of line infection than in other HPN-treated patients at Hope Hospital (0.52/1000 catheter days). Three patients died during the 13-yr period, none of causes related to their PN. Six were unable to manage their HPN regime themselves, mainly because of problems with hand function.

Although patient numbers were small, our findings suggest that HPN can be safely and successfully used long-term in patients with SSc and should be considered for patients unable to maintain their nutritional status because of severe gastrointestinal involvement. Impaired hand function should not preclude SSc patients from receiving HPN: family members or community nurses may be trained in the care of the HPN line.