Background: The objective of this study was to demonstrate the feasibility and associations with short-term outcomes of a medical nutrition therapy (MNT) intervention in patients with systemic scleroderma (SSc). Materials and Methods: Eighteen patients with SSc, gastrointestinal (GI) involvement, and unintentional weight loss were consented and recruited for a 6-week MNT intervention, in addition to their usual medical management. MNT emphasized increased calorie and protein intake, modified textures, and lifestyle modifications. Symptoms, anthropometrics, diet (24-hour recall), and body composition (dual-energy x-ray absorptiometry) were assessed pre- and postintervention. Sarcopenia was defined as appendicular lean height (ALH) for women <5.45 kg/m2 and for men <7.26 kg/m2. Descriptive, parametric, and nonparametric statistics were conducted. Results: Participants (n = 18) were predominantly white (78%), female (89%), malnourished (83%), and 51.3 ± 11.0 years of age with a body mass index of 22.6 ± 6.7 kg/m2. Significant decreases in nutrition symptom scores (12.8 vs 7.6, P < .05) and improvements in ALH (5.6 ± 0.8 vs 5.8 ± 0.8 kg/m2, respectively; P = .05) occurred pre- vs postintervention, respectively (n = 14). Sarcopenia was observed in 54% of participants at baseline and 39% at follow-up (P = .02). Caloric intake (1400 vs 1577 kcal/d, P = .12) and macronutrient distribution (ie, % fat, protein, carbohydrate) did not change significantly pre- vs postintervention, respectively. Conclusions: Individually tailored MNT can improve symptom burden and potentially ALH in patients with SSc involving the GI tract. This study underscores the clinical potential of multidisciplinary patient management and the need for larger nutrition intervention trials of longer duration in these patients.