REVIEW PAPER
Nutritional disorders and nutrition-related conditions: an underestimated clinical problem in systemic sclerosis
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Department of Rheumatology, Clinical Immunology, Geriatrics and Internal Medicine, Medical University of Gdańsk, Poland
Submission date: 2024-09-12
Final revision date: 2024-09-30
Acceptance date: 2024-10-02
Online publication date: 2024-11-09
Corresponding author
Anna Wojteczek
Department of Rheumatology, Clinical Immunology, Geriatrics and Internal Medicine, Medical University of Gdansk,
17 Smoluchowskiego St., 80-214 Gdansk, Poland
Reumatologia 2024;62(5):368-380
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ABSTRACT
Nutritional disorders are significant but often underestimated complications in patients with systemic sclerosis (SSc). The most prevalent nutritional disorders in SSc are malnutrition, affecting up to 62.5% of patients, and sarcopenia, with a frequency of up to 42%. Thus, clinical vigilance is recommended for the detection of eating disorders in SSc patients, particularly those with gastrointestinal involvement, cardiopulmonary complications, an advanced disease stage, and high disease activity. Nutritional treatment should be carefully tailored to the patients’ clinical condition to ensure that it effectively addresses their specific needs. Studies focusing on enteral nutrition in SSc patients demonstrate its effectiveness in stabilizing or improving nutritional status in malnourished patients. In severe cases, parenteral nutrition offers viable options to support patient health. The findings highlight the importance of early nutritional assessment and intervention in improving patient outcomes and suggest that individualized nutritional therapy can be a critical component of comprehensive care for SSc patients.
REFERENCES (72)
1.
Norman K, Pichard C, Lochs H, Pirlich M. Prognostic impact of disease-related malnutrition. Clin Nutr 2008; 27: 5–15, DOI: 10.1016/j.clnu.2007.10.007.
2.
Wollheim FA. Classification of systemic sclerosis. Visions and reality. Rheumatology (Oxford) 2005; 44: 1212–1216, DOI: 10.1093/rheumatology/keh671.
3.
Sobolewski P, Maślińska M, Wieczorek M, et al. Systemic sclerosis – multidisciplinary disease: clinical features and treatment. Rheumatology 2019; 57: 221–233, DOI: 10.5114/reum.2019.87619.
4.
Cederholm T, Barazzoni R, Austin P, et al. ESPEN guidelines on definitions and terminology of clinical nutrition. Clin Nutr 2017; 36: 49–64, DOI: 10.1016/j.clnu.2016.09.004.
5.
Skipper A, Ferguson M, Thompson K, Castellanos VH, Porcari J. Nutrition screening tools: an analysis of the evidence. JPEN J Parenter Enteral Nutr 2012; 36: 292–298, DOI: 10.1177/0148607111414023.
6.
Kondrup J, Allison SP, Elia M, et al.; Educational and Clinical Practice Committee, European Society of Parenteral and Enteral Nutrition (ESPEN). ESPEN guidelines for nutrition screening 2002. Clin Nutr 2003; 22: 415–421, DOI: 10.1016/s0261-5614(03)00098-0.
7.
Baron M, Hudson M, Steele R; Canadian Scleroderma Research Group. Malnutrition is common in systemic sclerosis: results from the Canadian scleroderma research group database. J Rheumatol 2009; 36: 2737–2743, DOI: 10.3899/jrheum.090694.
8.
Murtaugh MA, Frech TM. Nutritional status and gastrointestinal symptoms in systemic sclerosis patients. Clin Nutr 2013; 32: 130–135, DOI: 10.1016/j.clnu.2012.06.005.
9.
Ortiz-Santamaria V, Puig C, Soldevillla C, et al. Nutritional support in patients with systemic sclerosis. Reumatol Clin 2014; 10: 283–287, DOI: 10.1016/j.reuma.2013.12.011.
10.
Cereda E, Codullo V, Klersy C, et al. Disease-related nutritional risk and mortality in systemic sclerosis. Clin Nutr 2014; 33: 558–561, DOI: 10.1016/j.clnu.2013.08.010.
11.
Caimmi C, Caramaschi P, Venturini A, et al. Malnutrition and sarcopenia in a large cohort of patients with systemic sclerosis. Clin Rheumatol 2018; 37: 987–997, DOI: 10.1007/s10067-017-3932-y.
12.
Dupont R, Longué M, Galinier A, et al. Impact of micronutrient deficiency & malnutrition in systemic sclerosis: Cohort study and literature review. Autoimmun Rev 2018; 17: 1081–1089, DOI: 10.1016/j.autrev.2018.05.010.
13.
Preis E, Franz K, Siegert E, et al. The impact of malnutrition on quality of life in patients with systemic sclerosis. Eur J Clin Nutr 2018; 72: 504–510, DOI: 10.1038/s41430-018-0116-z.
14.
Türk İ, Cüzdan N, Çiftçi V, et al. Malnutrition, associated clinical factors, and depression in systemic sclerosis: a cross-sectional study. Clin Rheumatol 2020; 39: 57–67, DOI: 10.1007/s10067-019-04598-y.
15.
Yalcinkaya Y, Erturk Z, Unal AU, et al. The assessment of malnutrition and severity of gastrointestinal disease by using symptom-based questionnaires in systemic sclerosis: is it related to severe organ involvement or capillary rarefaction at microcirculation? Clin Exp Rheumatol 2020; 38 Suppl 125: 127–131.
16.
Molfino A, Gasperini ML, Gigante A, et al. Left Ventricular Mass Index as Potential Surrogate of Muscularity in Patients With Systemic Sclerosis Without Cardiovascular Disease. JPEN J Parenter Enteral Nutr 2021; 45: 1302–1308, DOI: 10.1002/jpen.1999.
17.
Hvas CL, Harrison E, Eriksen MK, et al. Nutritional status and predictors of weight loss in patients with systemic sclerosis. Clin Nutr ESPEN 2020; 40: 164–170, DOI: 10.1016/j.clnesp.2020.09.030.
18.
Pinheiro AC, Roque LCSC, Gonçalves RSG, et al. Nutritional risk in patients with systemic sclerosis. Clin Rheumatol 2020; 39: 295–297, DOI: 10.1007/s10067-019-04861-2.
19.
Rosato E, Gigante A, Gasperini ML, et al. Assessing Malnutrition in Systemic Sclerosis With Global Leadership Initiative on Malnutrition and European Society of Clinical Nutrition and Metabolism Criteria. JPEN J Parenter Enteral Nutr 2021; 45: 618–624, DOI: 10.1002/jpen.1872.
20.
Burlui AM, Cardoneanu A, Macovei LA, et al. Diet in Scleroderma: Is There a Need for Intervention? Diagnostics (Basel) 2021; 11: 2118, DOI: 10.3390/diagnostics11112118.
21.
Rosato E, Gigante A, Pellicano C, et al. Symptoms related to gastrointestinal tract involvement and low muscularity in systemic sclerosis. Clin Rheumatol 2022; 41: 1687–1696, DOI: 10.1007/s10067-022-06059-5.
22.
Rosato E, Gigante A, Pellicano C, et al. Phase angle, nutritional status, and mortality in systemic sclerosis: an exploratory pilot study. Nutrition 2023; 107: 111946, DOI: 10.1016/j.nut.2022.111946.
23.
Rivet V, Riviere S, Goulabchand R, et al. High prevalence of malnutrition in systemic sclerosis: Results from a French monocentric cross-sectional study. Nutrition 2023; 116: 112171, DOI: 10.1016/j.nut.2023.112171.
24.
Elia M, Stratton RJ. An analytic appraisal of nutrition screening tools supported by original data with particular reference to age. Nutrition 2012; 28: 477–494, DOI: 10.1016/j.nut.2011.11.009.
25.
Caporali R, Caccialanza R, Bonino C, et al. Disease-related malnutrition in outpatients with systemic sclerosis. Clin Nutr 2012; 31: 666–671, DOI: 10.1016/j.clnu.2012.02.010.
26.
Krause L, Becker MO, Brueckner CS, et al. Nutritional status as marker for disease activity and severity predicting mortality in patients with systemic sclerosis. Ann Rheum Dis 2010; 69: 1951–1957, DOI: 10.1136/ard.2009.123273.
27.
Rosato E, Gigante A, Gasperini ML, et al. Nutritional status measured by BMI is impaired and correlates with left ventricular mass in patients with systemic sclerosis. Nutrition 2014; 30: 204–209, DOI: 10.1016/j.nut.2013.07.025.
28.
Spanjer MJ, Bultink IEM, de van der Schueren MAE, Voskuyl AE. Prevalence of malnutrition and validation of bioelectrical impedance analysis for the assessment of body composition in patients with systemic sclerosis. Rheumatology (Oxford) 2017; 56: 1008–1012, DOI: 10.1093/rheumatology/kex014.
29.
Corallo C, Fioravanti A, Tenti S, et al. Sarcopenia in systemic sclerosis: the impact of nutritional, clinical, and laboratory features. Rheumatol Int 2019; 39: 1767–1775, DOI: 10.1007/s00296-019-04401-w.
30.
Wojteczek A, Dardzińska JA, Małgorzewicz S, et al. Prevalence of malnutrition in systemic sclerosis patients assessed by different diagnostic tools. Clin Rheumatol 2020; 39: 227–232, DOI: 10.1007/s10067-019-04810-z.
31.
Paolino S, Pacini G, Schenone C, et al. Nutritional Status and Bone Microarchitecture in a Cohort of Systemic Sclerosis Patients. Nutrients 2020; 12: 1632, DOI: 10.3390/nu12061632.
32.
Mękal D, Deptała A. Ocena stanu odżywienia chorych na twardzinę układową [Evaluation of nutritional status in patients with systemic sclerosis]. Pol Merkur Lekarski 2021; 49: 40–43.
33.
Rosato E, Gigante A, Colalillo A, et al. GLIM-diagnosed malnutrition predicts mortality and risk of hospitalization in systemic sclerosis: a retrospective study. Eur J Intern Med 2023; 117: 103–110, DOI: 10.1016/j.ejim.2023.07.017.
34.
Fairley JL, Hansen D, Quinlivan A, et al. Frequency and implications of malnutrition in systemic sclerosis. Rheumatology (Oxford) 2024; keae209, DOI: 10.1093/rheumatology/keae209.
35.
Wojteczek A, Dardzińska J, Ziętkiewicz M, et al. High-Protein Nutritional Supplements Improve Nutritional Status in Malnourished Patients with Systemic Sclerosis. Nutrients 2024; 16: 2622, DOI: 10.3390/nu16162622.
36.
Marighela TF, Genaro Pde S, Pinheiro MM, et al. Risk factors for body composition abnormalities in systemic sclerosis. Clin Rheumatol 2013; 32: 1037–1044, DOI: 10.1007/s10067-013-2235-1.
37.
Cederholm T, Bosaeus I, Barazzoni R, et al. Diagnostic criteria for malnutrition – An ESPEN Consensus Statement. Clin Nutr 2015; 34: 335–340, DOI: 10.1016/j.clnu.2015.03.001.
38.
Khalil SF, Mohktar MS, Ibrahim F. The theory and fundamentals of bioimpedance analysis in clinical status monitoring and diagnosis of diseases. Sensors (Basel) 2014; 14: 10895–10928, DOI: 10.3390/s140610895.
39.
Rosato E, Gigante A, Iacolare A, et al. Reduction of fat free mass index and phase angle is a risk factor for development digital ulcers in systemic sclerosis patients. Clin Rheumatol 2020; 39: 3693–3700, DOI: 10.1007/s10067-020-05141-0.
40.
Godala M, Gaszyńska E, Walczak K, Małecka-Wojciesko E. Evaluation of Albumin, Transferrin and Transthyretin in Inflammatory Bowel Disease Patients as Disease Activity and Nutritional Status Biomarkers. Nutrients 2023; 15: 3479, DOI: 10.3390/nu15153479.
41.
Codullo V, Cereda E, Klersy C, et al. Serum prealbumin is an independent predictor of mortality in systemic sclerosis outpatients. Rheumatology (Oxford) 2016; 55: 315–319, DOI: 10.1093/rheumatology/kev322.
42.
Valentini G, Bencivelli W, Bombardieri S, et al. European Scleroderma Study Group to define disease activity criteria for systemic sclerosis. III. Assessment of the construct validity of the preliminary activity criteria. Ann Rheum Dis 2003; 62: 901–903, DOI: 10.1136/ard.62.9.901.
43.
Gigante A, Gasperini ML, Iacolare A, et al. Phase angle in systemic sclerosis: a marker for pulmonary function and disease severity. Clin Rheumatol 2020; 39: 1699–1701, DOI: 10.1007/s10067-020-05034-2.
44.
Medsger TA Jr, Bombardieri S, Czirjak L, et al. Assessment of disease severity and prognosis. Clin Exp Rheumatol 2003; 21 (3 Suppl 29): S42–S46.
45.
Lim SL, Lin XH, Daniels L. Seven-Point Subjective Global Assessment Is More Time Sensitive Than Conventional Subjective Global Assessment in Detecting Nutrition Changes. JPEN J Parenter Enteral Nutr 2016; 40: 966–972, DOI: 10.1177/0148607115579938.
46.
Cederholm T, Jensen GL, Correia MITD, et al. GLIM criteria for the diagnosis of malnutrition – A consensus report from the global clinical nutrition community. J Cachexia Sarcopenia Muscle 2019; 10: 207–217, DOI: 10.1002/jcsm.12383.
47.
Cruz-Jentoft AJ, Baeyens JP, Bauer JM, et al. Sarcopenia: European consensus on definition and diagnosis: Report of the European Working Group on Sarcopenia in Older People. Age Ageing 2010; 39: 412–423, DOI: 10.1093/ageing/afq034.
48.
Cruz-Jentoft AJ, Sayer AA. Sarcopenia. Lancet 2019; 393: 2636–2646, DOI: 10.1016/S0140-6736(19)31138-9.
49.
Cruz-Jentoft AJ, Bahat G, Bauer J, et al. Sarcopenia: revised European consensus on definition and diagnosis. Age Ageing 2019; 48: 16–31, DOI: 10.1093/ageing/afy169.
50.
Siegert E, March C, Otten L, et al. Prevalence of sarcopenia in systemic sclerosis: assessing body composition and functional disability in patients with systemic sclerosis. Nutrition 2018; 55–56: 51–55, DOI: 10.1016/j.nut.2018.03.046.
51.
Sari A, Esme M, Aycicek GS, et al. Evaluating skeletal muscle mass with ultrasound in patients with systemic sclerosis. Nutrition 2021; 84: 110999, DOI: 10.1016/j.nut.2020.110999.
52.
Paolino S, Goegan F, Cimmino MA, et al. Advanced microvascular damage associated with occurence of sarcopenia in systemic sclerosis patients: results from a retrospective cohort study. Clin Exp Rheumatol 2020; 38 Suppl 125: 65–72.
53.
Hax V, do Espírito Santo RC, Dos Santos LP, et al. Practical screening tools for sarcopenia in patients with systemic sclerosis. PLoS One 2021; 16: e0245683, DOI: 10.1371/journal.pone.024568.
54.
Sangaroon A, Foocharoen C, Theerakulpisut D, et al. Prevalence and clinical association of sarcopenia among Thai patients with systemic sclerosis. Sci Rep 2022; 12: 18198, DOI: 10.1038/s41598-022-21914-w.
55.
Shafiee G, Keshtkar A, Soltani A, et al. Prevalence of sarcopenia in the world: a systematic review and meta-analysis of general population studies. J Diabetes Metab Disord 2017; 16: 21, DOI: 10.1186/s40200-017-0302-x.
56.
WHO Expert Consultation. Appropriate body-mass index for Asian populations and its implications for policy and intervention strategies. Lancet 2004; 363: 157–163, DOI: 10.1016/S0140-6736(03)15268-3.
57.
Donini LM, Busetto L, Bischoff SC, et al. Definition and Diagnostic Criteria for Sarcopenic Obesity: ESPEN and EASO Consensus Statement. Obes Facts 2022; 15: 321–335, DOI: 10.1159/000521241.
58.
Nguyen AD, McMahan ZH, Volkmann ER. Micronutrient Deficiencies in Systemic Sclerosis: A Scoping Review. Open Access Rheumatol 2022; 14: 309–327, DOI: 10.2147/OARRR.S354736.
59.
Harrison E, Herrick AL, McLaughlin JT, Lal S. Malnutrition in systemic sclerosis. Rheumatology (Oxford) 2012; 51: 1747–1756, DOI: 10.1093/rheumatology/kes160.
60.
Doerfler B, Allen TS, Southwood C, et al. Medical Nutrition Therapy for Patients With Advanced Systemic Sclerosis (MNT PASS): A Pilot Intervention Study. JPEN J Parenter Enteral Nutr 2017; 41: 678–684, DOI: 10.1177/0148607115597883.
61.
Low AHL, Teng GG, Pettersson S, et al. A double-blind randomized placebo-controlled trial of probiotics in systemic sclerosis associated gastrointestinal disease. Semin Arthritis Rheum 2019; 49: 411–419, DOI: 10.1016/j.semarthrit.2019.05.006.
62.
Marighela TF, Arismendi MI, Marvulle V, et al. Effect of probiotics on gastrointestinal symptoms and immune parameters in systemic sclerosis: a randomized placebo-controlled trial. Rheumatology (Oxford) 2019; 58: 1985–1990, DOI: 10.1093/rheumatology/kez160.
63.
Fynne L, Kruse A, Borre M, et al. Percutaneous endoscopic gastrostomy in patients with systemic sclerosis. Scand J Rheumatol 2010; 39: 266–268, DOI: 10.3109/03009740903468990.
64.
Falls AC, Wrigley C, Khanna SA. Progressive Systemic Sclerosis With Negative Antinuclear Antibodies and Absence of Raynaud’s Phenomenon: A Case Report and Literature Review. Cureus 2023; 15: e35663, DOI: 10.7759/cureus.35663.
65.
Stainforth J, Goodfield MD. Severe oropharyngeal deglutition abnormalities in a patient with systemic sclerosis, managed with a gastrostomy. Br J Dermatol 1994; 130: 682–683, DOI: 10.1111/j.1365-2133.1994.tb13124.x.
66.
Nunokawa T, Yokogawa N, Ohtsuka H, et al. Transgastric long tube placement following percutaneous endoscopic gastrostomy for severe chronic intestinal pseudo-obstruction related to systemic sclerosis. Mod Rheumatol 2015; 25: 958–961, DOI: 10.3109/14397595.2013.844385.
67.
Harrison E, Herrick AL, Dibb M, et al. Long-term outcome of patients with systemic sclerosis requiring home parenteral nutrition. Clin Nutr 2015; 34: 991–996, DOI: 10.1016/j.clnu.2014.11.002.
68.
Suzon B, Rivière S, Schiffmann A, et al. Long-term home parenteral nutrition in systemic sclerosis-related intestinal failure is feasible but unveils occult cardiac disease. Nutrition 2023; 110: 112009, DOI: 10.1016/j.nut.2023.112009.
69.
Levien DH, Fiallos F, Barone R, Taffet S. The use of cyclic home hyperalimentation for malabsorption in patients with scleroderma involving the small intestines. JPEN J Parenter Enteral Nutr 1985; 9: 623–625, DOI: 10.1177/0148607185009005623.
70.
Jawa H, Fernandes G, Saqui O, Allard JP. Home parenteral nutrition in patients with systemic sclerosis: a retrospective review of 12 cases. J Rheumatol 2012; 39: 1004–1007, DOI: 10.3899/jrheum.110896.
71.
Brown M, Teubner A, Shaffer J, Herrick AL. Home parenteral nutrition – an effective and safe long-term therapy for systemic sclerosis-related intestinal failure. Rheumatology (Oxford) 2008; 47: 176–179, DOI: 10.1093/rheumatology/kem329.
72.
Ng SC, Clements PJ, Berquist WE, Furst DE, Paulus HE. Home central venous hyperalimentation in fifteen patients with severe scleroderma bowel disease. Arthritis Rheum 1989; 32: 212–216, DOI: 10.1002/anr.1780320216.
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