Systemic sclerosis (SSc) is a connective tissue disorder characterized by inflammation and fibrosis of the skin, blood vessels and multiple internal organ involvement. The gastrointestinal tract is the second most affected organ, found in almost 90% of patients, and the disorder can occur along its entire length: esophagus, stomach, small intestine, colon and anorectal portion.1 About half of the patients have nausea, postprandial fullness, bloating and changes in bowel habits, and may be associated with loss of body weight.2 The impact of this disease on the digestive tract may contribute significantly to the deterioration of the nutritional state.3

It has been reported that the prevalence of malnutrition in these patients ranges from 15 to 30% according to several case series.4–6 To overcome this deficit, a panel of experts from Canada drew up recommendations to detect malnutrition and malabsorption in these patients.6 Malnutrition, along with all the symptoms associated with SS, has been associated with a poor perceived quality of life,5,7 which is lower than that reported for the general population.8 In addition, this patient population referred a higher incidence of depressive symptoms,9 although symptoms related to anxiety did not receive the same attention and could be a result to be considered.10

It is therefore necessary to identify patients at risk of malnutrition to apply measures of nutritional support and to evaluate their effectiveness on a physical, mental and perceived quality of life level.

Only general dietary recommendations symptoms as per the need of the patients have been described to date, and there are no studies assessing the benefits of nutritional intervention.

Based on the above, we evaluate whether the application of a nutritional support protocol11 in SS patients who visited the outpatient department of a community hospital improves their nutritional status, emotional state and quality of life.

72 ambulatory patients with SS (65 women and 7 men), were controled, 58 with limited SS and 14 with diffuse SS.

9 patients who met inclusion criteria (8 female and one male) with a mean age of 62.6±11.7 (51–80) years were included in the study. The clinical characteristics of the patients are described in Table 1. One-year follow-up was conducted. Of these, 6 patients had completed primary schooling and one, secondary studies. Four patients performed unskilled jobs, 2 had skilled jobs and one was a homemaker. Of the 9 patients, 4 had limited SS and 5 diffuse SS. Throughout the year follow-up one patient had pneumonia which resolved with antibiotics and 2 patients died from lung disease associated with SS.

Of the 9 patients included on initial visit 3 patients (33.3%) had a BMI below 20kg/m2, but none of them presented a BMI of less than 18.5kg/m2. Weight gain and BMI throughout the year of intervention was not statistically significant. One patient always maintained a BMI of 19kg/m2, and the BMI of the remaining patients was higher than 20 during the study. Table 2 shows the evolution of weight, BMI, energy and protein intake, estimation of nutritional requirements and the evolution of nutritional biochemistry.

At the initial visit, patients ate 86.8%±18.3% of their daily energy estimate and 97.5%±17.7% of protein needs. 33% of the patients ate less than 75% of their estimated energy requirements and protein intake in all was above 75%. At the initial visit 3 patients needed oral nutritional supplements, but we preferred to prescribe a basic tailored diet and evaluate them later. Thus, during follow-up visits 3 patients were prescribed oral nutritional supplements 500ml daily of a complete, polymer, and normoporteic normocaloric diet, which were dismissed by one patient.

In each of the evaluations of food logs we observed that 16.6% had an energy intake of less than 75% of daily energy estimate, not always in the same patient. Protein intake was less than 75% in one patient at 9 and 12 months. An increase in protein and energy intake during the dietary intervention was observed, but the differences were not statistically significant.

Regarding the biochemical parameters evaluated, 6 patients (54.5%) had low hemoglobin levels, but just 2 patients had low ferritin levels (18.3%). The mean hemoglobin levels improved during the intervention, although the differences were not significant. Two patients received oral iron supplementation. It was observed that the average levels of ferritin decreased, though not statistically significant, due to a patient who always had low values.

Of the 9 patients, none had to start folic acid, vitamin B12, vitamin A, zinc due to coagulation disorder or due to deficiency. 54% were deficient in vitamin D, and in 18% this had not been determined. Of the patients with deficiency of vitamin D, 16.6% were severe (<10ng/ml), in 66.6% it was important (10–20ng/ml) and 16.6% were moderate (20–30ng/ml).

Three patients received supplementation with calcium and vitamin D (600–800IU/day) and 3 patients were prescribed higher doses of the same. The evolution of the average levels of vitamin D improved slightly, but at the end of the study all had insufficient vitamin D.

Two patients of the 9 who had albumin determinations showed low levels: one at the initial visit (11%), which improved during the dietary intervention, and in the other patient this was detected in the 3 month control.

The psychological evaluation was possible in 5 of the 9 patients, and evaluation of quality of life, in 7 of the 9 patients. At baseline, the mean anxiety score was 8.00±2.71, and depression, 8.8±8.19 Of the patients evaluated, 60% had a score greater than 7 for anxiety and at 40% for depression score.

Table 3 shows the mean scores of the SF-36, where it is noteworthy that all patients assessed scored below the criteria of normality in all dimensions, both at baseline and at the end of the intervention. Throughout the operation, no statistically significant differences were observed.

This work evaluates the impact of a nutritional intervention in patients with SS at risk of malnutrition and their nutritional status and quality of life.

Several studies have shown the high prevalence of malnutrition in these patients,4–6 but there are no studies that assess the impact of a structured nutritional intervention after one year of follow-up. In the present series, 12.5% of patients with SS were candidates for nutritional intervention, using the MUST screening method. The prevalence is similar to that described in the literature.

At the initial visit, when the nutritional assessment was performed, we found that three patients had an energy intake of less than 75% of estimated needs. In all cases the intake covered over 50% of the nutritional requirements. In our sample, the prevalence of inadequate energy intake was highly lower than that reported by Krause et al.,17 as was the prevalence of patients requiring nutritional support, either through oral intake or artificial nutrition. The discrepancy could be explained by the fact that both studies have used different methods to estimate the requirements and energy intake. It has been reported that if the food is enriched, this improves energy and protein intake between 23 and 26%, approximately, without affecting hunger before the meal or the desire to eat, leading us to prescribe oral nutritional supplements when intake is less than 75% of requirements.18 Although these 3 patients had such an indication, we preferred to start a basic tailored diet and evaluate the patient at the next control visit. In the follow-up visit we prescribed an oral nutritional supplement to 3 patients, although one of them refused.

The recommendations of the panel of American experts did not include the determination of plasma albumin routinely in nutritional screening, since the type of malnutrition observed in these patients is mainly caloric.6 In our series, only two patients had low plasma albumin levels. One of them was detected in the second follow-up visit; it was a patient who breached the recommendations given and did not come for further control visits. The other patient had, without a context of infection, low albumin at the initial visit, reflecting protein-energy malnutrition. This patient improved with nutritional intervention, but died after 18 months from the start of the intervention due to multisystem involvement. In patients with SS, the existence of an association between mortality and BMI less than 18.519kg/m2 has been described, as it has with specific body composition determined by bioelectrical impedance.17 However, the detection of hypoalbuminemia surely confers an increased morbidity and thereby leads to death, as suggested above.4 In this sense, another patient died of respiratory infection in the setting of severe pulmonary involvement secondary to SS and showed initial albumin levels at the lower limit of normal (36mg/dl).

Iron deficiency anemia and vitamin D deficiency were attributable to complications associated to SS with nutritional deficit observed, such as gastrointestinal vascular ectasia and malabsorption syndrome. No vitamin A, vitamin B12, folic acid deficits or bleeding disorders were observed. The prevalence of vitamin D deficiency observed is similar to that described in a cross-sectional study in patients with SS performed in Spain.20 At the end of the period of nutritional intervention, all had insufficient vitamin D. This finding could be explained by other factors such as malabsorption, physical inactivity and impaired dermal activation; low hydroxylation of vitamin D could contribute21 as well as polypharmacy characteristic of such patients, preventing both the prescription and its completion.

At the end of the intervention, according to the new diagnostic criteria for malnutrition22 and with the techniques available in our environment (body weight gain, food intake and the presence of fluid retention), only one patient had malnutrition, and had specifically rejected oral nutritional supplements.

The results observed in the psychopathology and quality of life questionnaires showed a high prevalence of anxiety and depression, lower than that reported in the general population2,8 for physical and mental items. Although the patients reported feeling better, statistical analysis of the results of the tests and questionnaires did not show significant improvement throughout the intervention. These results may be due to the small sample size, the high incidence of anxiety and depression that can interfere with proper adhesion to therapeutic measures associated to the loss of 2 patients during follow-up, the presence of concomitant diseases, clinical worsening during cold periods reported by the patients and items not reflected in the questionnaires, among other possibilities.

The risk of malnutrition in patients with SS affects a striking percentage of patients. In our series it reached 12.5% of patients. The dietary intervention was able to maintain or increase body weight, and energy and protein intake. The iron and vitamin D deficiency improved with supplementation. The psychological assessment and questionnaire of life test did not reflect a statistically significant improvement, although the patients reported feeling better. Longitudinal studies are needed with larger numbers of patients to assess the efficacy of this intervention beyond nutritional outcomes.

The authors have no conflicts of interest.