RESUMEN
BACKGROUND & AIMS: Scleroderma is a multi-system disease that causes hardening of connective tissue. The gastrointestinal (GI) tract is affected in 90% of patients, which may cause nutritional decline. Due to the rarity of the disease, current nutritional guidelines in scleroderma are extrapolated from evidence in other chronic diseases. This systematic review examines the effects of oral nutrition supplements (ONS), enteral nutrition (EN) and parenteral nutrition (PN) on both clinical and nutritional outcomes of scleroderma patients. METHOD: Three separate systematic searches for scleroderma and ONS, EN, and PN were performed. The searches were conducted using EMBASE, PubMed and Web of Science databases. RESULTS: A total of 9 studies (ONS: 2, EN: 1 and PN: 6) met the inclusion and exclusion criteria and were included in the review. All patients had scleroderma and were malnourished or at risk of malnutrition [weighted average age: 53 years, Body Mass Index (BMI): 19 kg/m2]. Artificial nutrition support was shown to be an effective therapy for preventing nutritional decline and reversing malnutrition but had no impact on disease progression. Mean BMI increased with home parenteral nutrition (HPN) and EN, from 15 kg/m2 to 21.0 kg/m2. Weight was maintained with ONS, and sarcopenia decreased. Only HPN positively impacted quality of life and GI symptoms, with complication rates similar to patients with other indications. CONCLUSION: Scleroderma patients should be routinely screened for malnutrition. Malnutrition is treated in a stepwise manner, starting with ONS, then EN and finally PN, based on GI sufficiency and tolerance of the patient. HPN is an effective therapy for patients with advanced disease and intestinal failure. However, larger, long-term, prospective studies for each nutritional therapy are required to make firm conclusions.