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

Objectives. 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. Methods. 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. Results. 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 13851 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. Conclusions. 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. Background Approximately 90% of patients with the multisystem connective tissue disease SSc develop gastrointestinal involvement of their disease, although many are asymptomatic [1, 2]. Gastrointestinal problems occur in both the lcSSc and dcSSc disease subtypes [3]. Any part of the gastrointestinal tract may become involved and an autopsy study showed that approximately half of the patients have histological involvement of their small bowel [4]. A small minority of patients develop intestinal failure. The usual scenario in this minority is that small intestinal involvement, with impaired peristalsis, results in bowel dilatation and stasis that in turn lead to bacterial overgrowth, malabsorption, and sometimes pseudoobstruction. Gastrointestinal involvement in SSc can therefore be life-threatening: of 264 patients with dcSSc from 29 centres, with an average duration of follow-up of 5.2 yrs, 13 (5%) died of gastrointestinal disease, representing 15% of deaths definitely related to SSc [5]. When a patient is unable to maintain his/her nutritional status, as in SSc-related intestinal failure, parenteral nutrition (PN) may be required to prevent, or correct, malnutrition. Other forms of artificial feeding, including oral supplementation, naso-jejunal feeding and feeding via percutaneous enterogastrostomy (PEG)/ percutaneous jejunostomy tubes may be tried before introducing PN. Complications of PN administration include central venous catheter infections, occlusions and fractures of the central line, hepatic disease and osteoporosis. If the indication for PN suggests that long-term treatment will be required, as in SSc, home parenteral nutrition (HPN) may be considered. PN is a potentially life-saving treatment but training the patient as carer for the central line for HPN may pose particular problems in SSc because of concomitant problems including impaired hand function and cardiorespiratory disease. The aim of this retrospective study was to examine the outcome in patients with SSc requiring PN, and to compare their clinical characteristics with those of other SSc patients and of patients requiring PN/HPN for other conditions.