Nutritional status in chronic obstructive pulmonary disease and systemic sclerosis: two systemic diseases involving the respiratory system.

This study aimed to assess and compare the nutritional status and life quality of patients with chronic obstructive pulmonary disease (COPD) and systemic sclerosis (SSc). Thirty patients with stable COPD and 32 patients with SSc were examined. In all patients, the following parameters were measured: fat mass, fat-free mass, total body water, FEV1, and blood gases. COPD patients' life quality was assessed with St. George's Respiratory Questionnaire, and in SSc patients with a Quality of Life Questionnaire. The results show that among COPD patients 13% had normal body weight, 60% were obese, and 27% were overweight. In SSc patients, 59% had normal body weight, 31% were overweight, 1 patient was obese, and 2 were underweight. The mean life quality score in COPD patients was 57.3±16.5, while that in SSc patients was 35.8±18.2. COPD patients had a statistically significant lower life quality than SSc patients. The mean value of FEV1 was 45.5±12.2% pred. in COPD patients, and 86.8±21.2% pred. in the SSc group. We conclude that nutritional disorders are more frequent in COPD patients compared to those with SSc.

High-Protein Nutritional Supplements Improve Nutritional Status in Malnourished Patients with Systemic Sclerosis.

(1) Background: Impaired nutritional status in systemic sclerosis (SSc) is prevalent. (2) Objective: This study aimed to identify pre-cachexia and malnutrition in SSc patients and to estimate the effectiveness of a high-protein oral nutritional supplement (ONS) in improving their nutritional status. (3) Materials and methods: The SSc population comprised 56 patients and a control group of 49 healthy persons. After a baseline clinical evaluation, bioelectrical impedance analysis (BIA), and laboratory tests, SSc patients were divided into well-nourished, pre-cachectic, and malnourished categories. SSc patients with a nutritional disbalance received a high-protein ONS once daily for 3 months. Patients were reassessed at 3 and 12 months after inclusion in the study. (4) Results: SSc patients, in comparison to the control group, had a significantly lower seven-point SGA value [6(0) vs. 7(1), p < 0.001)], lean tissue mass [LTM, 35.1 (10.5) vs. 40.1 (10.10), p = 0.008], and lean tissue index [LTI, 13.5 (3) vs. 14.9 (4), p = 0.009]. Of the 56 SSc patients, 40 (71.4%) were well nourished, 5 (8.9%) had pre-cachexia, and 11 (19.7%) were malnourished. A high-protein ONS in the pre-cachexia group stabilized the SGA value, anthropometric measurements, and BIA after 3 and 12 months. In malnourished patients, it significantly improved the SGA value [5(0) vs. 6(0), p = 0.002], LTI [12.1 (2.1) vs. 12.7 (3.2), p = 0.021] and LTM [31.1 (7.7) vs. 35.1 (9.1), p = 0.021], and that effect remained stable at 12 months. (5) Conclusion: Malnutrition is a common complication of SSc that can be improved with nutritional intervention.

Role of Vitamin D in Systemic Sclerosis: A Systematic Literature Review.

BACKGROUND: Systemic sclerosis (SSc) is a chronic multisystem autoimmune condition defined by a complex pathobiology, comprising excessive fibrosis of skin and internal organs, peripheral vasculopathy with endothelial cell dysfunction, inadequate vascular repair and neovascularization, and aberrant immunity. Vitamin D is a steroid hormone with pleiotropic effects beyond its traditional role in calcium and bone homeostasis. Since vitamin D has immunomodulatory, cardioprotective, and antifibrotic properties, it could potentially interfere with SSc pathogenesis. Suboptimal vitamin D levels are classically recognized in scleroderma, irrespective of clinical and serological phenotype. AIM: This systematic review is aimed at investigating and clarifying the role of vitamin D in SSc and emphasizing the association of vitamin D status with different clinical settings. METHODS AND RESULTS: A systematic online search was performed, using PubMed databases to collect articles on the topic of vitamin D in SSc. The final analysis included 40 eligible articles. CONCLUSIONS: Hypovitaminosis D is common in SSc patients and could be associated with clinical and serologic patterns of the disease. Intervention for low serum vitamin D levels in SSc pathogenesis remains controversial, as well as the significance of vitamin D supplementation in such patients.

Pregnancy and childbirth after treatment with autologous hematopoietic stem cell transplantation for severe systemic sclerosis requiring parenteral nutrition. Ethical issues.

We report a pregnancy in a patient who had undergone autologous hematopoietic stem cell transplantation (AHSCT) for diffuse cutaneous systemic sclerosis (SSc). SSc onset was at age 25, with Raynaud phenomenon and evolved to include diffuse cutaneous, arthritis, pulmonary fibrosis and extensive gastrointestinal involvement. AHSCT (cyclophosphamide/ATG for conditioning) was performed four years later with improvement of all features apart from the gastrointestinal symptoms requiring parenteral nutrition (PN). Forty months after AHSCT, she had a spontaneous miscarriage necessitating curettage. Despite advice to avoid pregnancy because of poor nutritional status and recurring catheter infections from her PN, she fell pregnant one year later. The pregnancy proceeded normally and she delivered at 34 weeks, under cesarean section. The baby girl, (1990g and 4 APGAR score) after initial respiratory distress, is now 4 years old with normal growth and development. Unfortunately, the patient died early in 2008 due to severe disease progression terminating with gastrointestinal obstruction and pericarditis. This first report of a successful pregnancy in a patient with diffuse SSc treated by AHSCT illustrates that despite the possibility for a normal pregnancy, the decision to do so includes aspects of maternal prognosis.

Medical Nutrition Therapy for Patients With Advanced Systemic Sclerosis (MNT PASS): A Pilot Intervention Study.

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.

Novel lung imaging biomarkers and skin gene expression subsetting in dasatinib treatment of systemic sclerosis-associated interstitial lung disease.

BACKGROUND: There are no effective treatments or validated clinical response markers in systemic sclerosis (SSc). We assessed imaging biomarkers and performed gene expression profiling in a single-arm open-label clinical trial of tyrosine kinase inhibitor dasatinib in patients with SSc-associated interstitial lung disease (SSc-ILD). METHODS: Primary objectives were safety and pharmacokinetics. Secondary outcomes included clinical assessments, quantitative high-resolution computed tomography (HRCT) of the chest, serum biomarker assays and skin biopsy-based gene expression subset assignments. Clinical response was defined as decrease of >5 or >20% from baseline in the modified Rodnan Skin Score (MRSS). Pulmonary function was assessed at baseline and day 169. RESULTS: Dasatinib was well-tolerated in 31 patients receiving drug for a median of nine months. No significant changes in clinical assessments or serum biomarkers were seen at six months. By quantitative HRCT, 65% of patients showed no progression of lung fibrosis, and 39% showed no progression of total ILD. Among 12 subjects with available baseline and post-treatment skin biopsies, three were improvers and nine were non-improvers. Improvers mapped to the fibroproliferative or normal-like subsets, while seven out of nine non-improvers were in the inflammatory subset (p = 0.0455). Improvers showed stability in forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO), while both measures showed a decline in non-improvers (p = 0.1289 and p = 0.0195, respectively). Inflammatory gene expression subset was associated with higher baseline HRCT score (p = 0.0556). Non-improvers showed significant increase in lung fibrosis (p = 0.0313). CONCLUSIONS: In patients with SSc-ILD dasatinib treatment was associated with acceptable safety profile but no significant clinical efficacy. Patients in the inflammatory gene expression subset showed increase in skin fibrosis, decreasing pulmonary function and worsening lung fibrosis during the study. These findings suggest that target tissue-specific gene expression analyses can help match patients and therapeutic interventions in heterogeneous diseases such as SSc, and quantitative HRCT is useful for assessing clinical outcomes. TRIAL REGISTRATION: Clinicaltrials.gov NCT00764309.

Nutritional support in patients with systemic sclerosis.

UNLABELLED: Systemic sclerosis (SSc) is a chronic multisystem autoimmune disease which involves the gastrointestinal tract in about 90% of cases. It may contribute to nutritional deterioration. OBJECTIVE: To assess whether the application of a nutritional support protocol to these patients could improve their nutritional status and quality of life. METHODS: Single center prospective study, performed on an outpatient basis, in a county hospital. The Malnutrition Universal Screening Tool (MUST) was used to screen risk for malnutrition. Health questionnaire SF-36 and the Hospital Anxiety and Depression Scale were used to assess quality of life and psychopathology respectively. Weight, height, energy and protein requirements, macronutrient intake and nutritional biochemical parameters were evaluated. Nutritional intervention was performed in patients at risk for malnutrition. RESULTS: Of the 72 patients, 12.5% were at risk for malnutrition. Iron deficiency anemia (18.35%) and vitamin D deficiency (54%) were the most frequently observed nutritional deficits. The questionnaires on psychopathology and quality of life showed a high prevalence of anxiety and depression, and lower level poor quality of life in the physical and mental component. No significant improvements were observed in the weight, food intake, nutritional biochemical parameters, psychopathology and quality of life follow-up. CONCLUSIONS: Dietary intervention was able to maintain body weight and food intake. Iron deficiency anemia and vitamin D deficiency improved with iron and vitamine D supplements. No deterioration was observed in psychological assessment or quality of life. Studies with larger numbers of patients are needed to assess the efficacy of this intervention.

Home parenteral nutrition in patients with systemic sclerosis: a retrospective review of 12 cases.

OBJECTIVE: To evaluate the effect of home total parenteral nutrition (HTPN) on the nutritional and functional status of patients with systemic sclerosis (SSc). METHODS: Retrospective review of SSc patients from the HTPN database at the University Health Network, Toronto, Canada, from 1998 to 2010. RESULTS: In total 12 subjects (9 females, 3 males) with SSc were identified. At initiation of HTPN, the age was 48.75 ± 3.94 years and the median body mass index was 16.8 kg/m(2) (range 12.3-21.3). This significantly increased to 18.3 kg/m(2) (17.0-21.7) at 6 months and to 19.7 kg/m(2) (17.0-24.6) at 12 months. The mean Karnofsky score at baseline was 58.00 ± 3.27, and the score at 12 months of 39.00 ± 13.29 did not change significantly. CONCLUSION: HTPN improved the nutritional status of these patients with SSc but it did not improve their functional status.

Soporte nutricional a pacientes con esclerosis sistémica / Nutritional support in patients with systemic sclerosis

La esclerosis sistémica (ES) es una enfermedad autoinmune sistémica crónica que en cerca del 90% de los casos afecta al tracto gastrointestinal. Se estima que dicha alteración puede contribuir al deterioro nutricional. Objetivo. Evaluar si la aplicación de un protocolo de soporte nutricional a dichos pacientes mejora su estado nutricional y su calidad de vida. Método. Estudio prospectivo unicéntrico realizado en consultas externas de un hospital comarcal. Se utilizó el test MUST para el cribado de malnutrición. El cuestionario de salud SF-36 y el de Hospital Anxiety and Depression Scale se utilizaron para la valoración de la calidad de vida y psicopatológica, respectivamente. Se determinaron: el peso, la talla, las necesidades energéticas y proteicas, la ingesta de macronutrientes y los parámetros bioquímicos nutricionales. Se realizó intervención nutricional a los pacientes con riesgo. Resultados. De los 72 pacientes, el 12,5% tenían riesgo de malnutrición. La anemia ferropénica (18,35%) y el déficit de vitamina D (54%) fueron los déficits nutricionales más observados. Los cuestionarios de psicopatología y calidad de vida indicaron elevada prevalencia de ansiedad y depresión, y puntuaciones más bajas en las dimensiones física y mental según el SF-36. No se evidenciaron mejoras significativas en la evolución del peso, en la ingesta alimentaria ni en los parámetros bioquímicos nutricionales, psicopatológicos ni de calidad de vida. Conclusiones. La intervención dietética consiguió mantener el peso corporal y la ingesta energética y proteica. Los déficit de hierro y de vitamina D mejoraron con suplementación. No se observó un deterioro en la valoración psicológica ni en la calidad de vida. Se precisan estudios con mayor número de pacientes para valorar la eficacia de dicha intervención (AU)

Systemic sclerosis (SSc) is a chronic multisystem autoimmune disease which involves the gastrointestinal tract in about 90% of cases. It may contribute to nutritional deterioration. Objective: To assess whether the application of a nutritional support protocol to these patients could improve their nutritional status and quality of life. Methods: Single center prospective study, performed on an outpatient basis, in a county hospital. The Malnutrition Universal Screening Tool (MUST) was used to screen risk for malnutrition. Health questionnaire SF-36 and the Hospital Anxiety and Depression Scale were used to assess quality of life and psychopathology respectively. Weight, height, energy and protein requirements, macronutrient intake and nutritional biochemical parameters were evaluated. Nutritional intervention was performed in patients at risk for malnutrition. Results: Of the 72 patients, 12.5% were at risk for malnutrition. Iron deficiency anemia (18.35%) and vitamin D deficiency (54%) were the most frequently observed nutritional deficits. The questionnaires on psychopathology and quality of life showed a high prevalence of anxiety and depression, and lower level poor quality of life in the physical and mental component. No significant improvements were observed in the weight, food intake, nutritional biochemical parameters, psychopathology and quality of life follow-up. Conclusions: Dietary intervention was able to maintain body weight and food intake. Iron deficiency anemia and vitamin D deficiency improved with iron and vitamine D supplements. No deterioration was observed in psychological assessment or quality of life. Studies with larger numbers of patients are needed to assess the efficacy of this intervention (AU)

Dietary intake of micronutrient antioxidants in relation to blood levels in patients with systemic sclerosis.

OBJECTIVE: To document habitual intakes of micronutrient antioxidants in patients with systemic sclerosis (SSc) in light of studies reporting subnormal levels of ascorbate and selenium in this patient group. METHODS: Dietary intakes of vitamin C, selenium, alpha-tocopherol, beta-carotene, and sulfur amino acid precursors of glutathione were assessed using the 7 day weighed record in 12 patients with SSc and in 12 healthy control subjects. The intakes of the first 4 substances were examined in relation to plasma/serum levels, while intakes of sulfur amino acids were examined in relation to urinary inorganic sulfate. RESULTS: Antioxidant and sulfur amino acid intakes were similar in patients and controls, although the patients had lower levels of selenium (median 74 compared to 87 milligrams in controls; p = 0.014) and of vitamin C in plasma (median 6.0 compared to 11.1 milligrams/l in controls; p = 0.08). Inorganic sulfate concentration in urine was similar in patients and controls. CONCLUSION: Our results suggest that reduced blood levels of the water soluble antioxidants selenium and ascorbic acid in patients with SSc are not due to dietary deficiency. Other explanations must therefore be sought.

Nutrición en la esclerosis sistémica / Nutrition in systemic sclerosis

La esclerosis sistémica es una enfermedad del tejido conectivo caracterizada por inflamación y fibrosis de múltiples órganos (piel, aparato digestivo, pulmón, riñón y corazón). Después de la piel, el órgano más afectado, con una frecuencia del 75 al 90%, es el tracto gastrointestinal. La afectación del tracto gastrointestinal se manifiesta por la aparición de disfagia orofaríngea, disfagia esofágica, reflujo gastroesofágico, gastroparesia, seudoobstrucción, sobrecrecimiento bacteriano y malabsorción intestinal, estreñimiento, diarrea y/o incontinencia fecal. Estas afectaciones condicionan la ingesta alimentaria y la absorción intestinal y conducen a la aparición progresiva de deficiencias nutricionales. Alrededor de un 30% de los pacientes con esclerosis sistémica presentan un riesgo de malnutrición. En el 5-10%, los trastornos gastrointestinales son la principal causa de muerte. Las estrategias terapéuticas existentes en la actualidad son limitadas y están dirigidas a reducir la sintomatología clínica. El manejo multidisciplinar de dichos pacientes, que incluya la intervención nutricional, contribuye a mejorar la sintomatología gastrointestinal, además de evitar la malnutrición, la morbilidad y aumentar la calidad de vida (AU)

Systemic sclerosis is a connective tissue disease characterized by inflammation and fibrosis of multiple organs (skin, gastrointestinal tract, lung, kidney and heart). After the skin, the organ most affected with a frequency of 75 to 90%, the gastrointestinal tract is more often involved. Gastrointestinal tract involvement is manifested by the appearance of oropharyngeal dysphagia, esophageal dysphagia, gastroesophageal reflux, gastroparesis, pseudo-obstruction, bacterial overgrowth and intestinal malabsorption, constipation, diarrhea and/or fecal incontinence. These effects influence food intake and intestinal absorption leading to the gradual emergence of nutritional deficiencies. About 30% of patients with systemic sclerosis are at risk of malnutrition. In 5-10%, gastrointestinal disorders are the leading cause of death. Therapeutic strategies currently available are limited and aimed at reducing clinical symptoms. The multidisciplinary management of these patients, including nutritional intervention, helps improve gastrointestinal symptoms, and avoid malnutrition, morbidity and improve quality of life (AU)