Download PDF
Arthritis und Rheuma 2025; 45(02): 98-109
DOI: 10.1055/a-2438-9227
Schwerpunkt

Gastrointestinale Manifestationen bei systemischer Sklerose

Pascal van Wijnen
1   Campus Kerckhoff, Justus-Liebig-Universität Gießen, Abt. für Rheumatologie, Klinische Immunologie, Osteologie und Physikalische Medizin, Bad Nauheim
,
Ulf Müller-Ladner
1   Campus Kerckhoff, Justus-Liebig-Universität Gießen, Abt. für Rheumatologie, Klinische Immunologie, Osteologie und Physikalische Medizin, Bad Nauheim
,
Nils Schulz
1   Campus Kerckhoff, Justus-Liebig-Universität Gießen, Abt. für Rheumatologie, Klinische Immunologie, Osteologie und Physikalische Medizin, Bad Nauheim
,
Christopher Hackenberg
2   Agaplesion Markus Krankenhaus, Medizinische Klinik I (Gastroenterologie, Hepatologie, Onkologie, Infektiologie, Pneumologie), Frankfurt am Main
,
Philipp Klemm
1   Campus Kerckhoff, Justus-Liebig-Universität Gießen, Abt. für Rheumatologie, Klinische Immunologie, Osteologie und Physikalische Medizin, Bad Nauheim
› Author Affiliations
› Further Information
Also available at
    Permissions and Reprints

Zusammenfassung

Eine gastrointestinale Beteiligung bei systemischer Sklerose betrifft ca. 90 % der Patienten und ist maßgeblich bedingt durch fibrosierende Prozesse und Vaskulopathie. Diese führen zu Beschwerden, die von Dysphagie über Reflux, Meteorismus und Obstipation bis zu Blutungen reichen. Neben einer ausführlichen Anamnese sind oftmals eine weitergehende Abklärung mittels endoskopischer Untersuchungen (Ösophagogastroduodenoskopie und Koloskopie) sowie Schnittbildgebung notwendig.

Therapeutisch ist zunächst eine Anpassung der Ernährung sinnvoll. Die medikamentöse Therapie ist bislang rein symptomatisch, sodass neben Protonenpumpenhemmern oftmals Prokinetika, Laxanzien und selten Antibiotika vonnöten sind. Bei Gefäßektasien oder gastrointestinalen Blutungen kommen zudem endoskopische Interventionen zum Einsatz.

Schlüsselwörter

Systemische Sklerose - gastrointestinale Symptome - Dysphagie - Reflux - Obstipation - SIBO - IPO


Publication History

Article published online:
15 April 2025

© 2025. Thieme. All rights reserved.

Georg Thieme Verlag KG
Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

 

  • Literatur

  • 1 Van Den Hoogen F, Khanna D, Fransen J. et al. 2013 classification criteria for systemic sclerosis: An american college of rheumatology/European league against rheumatism collaborative initiative. Arthritis Rheum 2013; 65: 2737-2747
    CrossrefPubMedSearch in Google Scholar
  • 2 Bairkdar M, Rossides M, Westerlind H. et al. Incidence and prevalence of systemic sclerosis globally: A comprehensive systematic review and meta-analysis. Rheumatology (United Kingdom) 2021; 60: 3121-3133
    Search in Google Scholar
  • 3 Westerlind H, Bairkdar M, Gunnarsson K. et al. Incidence and prevalence of systemic sclerosis in Sweden, 2004–2015, a register-based study. Semin Arthritis Rheum 2022; 53: 151978
    CrossrefPubMedSearch in Google Scholar
  • 4 Di Maggio G, Confalonieri P, Salton F. et al. Biomarkers in Systemic Sclerosis: An Overview. Curr Issues Mol Biol 2023; 45: 7775-7802
    CrossrefPubMedSearch in Google Scholar
  • 5 Kucharz EJ, Kopeć-Mędrek M. Systemic sclerosis sine scleroderma. Adv Clin Exp Med 2017; 26: 875-880
    CrossrefPubMedSearch in Google Scholar
  • 6 De Almeida Chaves S, Porel T, Mounié M. et al. Sine scleroderma, limited cutaneous, and diffused cutaneous systemic sclerosis survival and predictors of mortality. Arthritis Res Ther 2021; 23: 295
    CrossrefPubMedSearch in Google Scholar
  • 7 Nassar M, Ghernautan V, Nso N. et al. Gastrointestinal involvement in systemic sclerosis: An updated review. Medicine (United States) 2022; 101: E31780
    Search in Google Scholar
  • 8 Schmeiser T, Saar P, Jin D. et al. Profile of gastrointestinal involvement in patients with systemic sclerosis. Rheumatol Int 2012; 32: 2471-2478
    CrossrefPubMedSearch in Google Scholar
  • 9 Shreiner AB, Murray C, Denton C. et al. Gastrointestinal manifestations of systemic sclerosis. J Scleroderma Relat Disord 2016; 1: 247-256
    CrossrefPubMedSearch in Google Scholar
  • 10 Luquez-Mindiola A, Atuesta AJ, Gómez-Aldana AJ. Gastrointestinal manifestations of systemic sclerosis: An updated review. World J Clin Cases 2021; 9: 6201-6217
    CrossrefPubMedSearch in Google Scholar
  • 11 Volkmann ER, McMahan Z. Gastrointestinal involvement in systemic sclerosis: pathogenesis, assessment and treatment. Curr Opin Rheumatol 2022; 34: 328-336
    CrossrefPubMedSearch in Google Scholar
  • 12 McFarlane IM, Bhamra MS, Kreps A. et al. Gastrointestinal Manifestations of Systemic Sclerosis. Rheumatology 2018; 8: 235
    CrossrefPubMedSearch in Google Scholar
  • 13 Freire M, Simeón Aznar CP. Clinical and epidemiological differences between men and women with systemic sclerosis: a study in a Spanish systemic sclerosis cohort and literature review. Clin Exp Rheumatol 2017; 35 (Suppl. 106) 89-97
    PubMedSearch in Google Scholar
  • 14 Derk CT, Rasheed M, Spiegel JR. et al. Increased Incidence of Carcinoma of the Tongue in Patients with Systemic Sclerosis. J Rheumatol 2005; 32: 637-641
    PubMedSearch in Google Scholar
  • 15 Pulumati A, Lin R, Elman SA. Therapeutic Promise of Hyaluronidase in Systemic Sclerosis: A Systematic Review. Dermatol Pract Concept 2024; 14: e2024163
    PubMedSearch in Google Scholar
  • 16 Wunderlin C, Napp S, Siegwart D. et al. Interdisziplinäre Therapieansätze der systemischen Sklerose. Rheuma Plus 2024; 23: 211-217
    CrossrefSearch in Google Scholar
  • 17 Parodis I, Girard-Guyonvarc'h C, Arnaud L. et al. EULAR recommendations for the non-pharmacological management of systemic lupus erythematosus and systemic sclerosis. Ann Rheum Dis 2024; 83: 720-729
    CrossrefPubMedSearch in Google Scholar
  • 18 Arif T, Masood Q, Singh J. et al. Assessment of esophageal involvement in systemic sclerosis and morphea (localized scleroderma) by clinical, endoscopic, manometric and pH metric features: A prospective comparative hospital based study. BMC Gastroenterol 2015; 15: 24
    CrossrefPubMedSearch in Google Scholar
  • 19 Petcu A, Ghib L, Grad S. et al. Upper gastrointestinal involvement in systemic sclerosis: Findings in a real-life setting. Exp Ther Med 2019; 18: 5095-5100
    CrossrefPubMedSearch in Google Scholar
  • 20 Panopoulos S, Bournia V, Karamanolis G. et al. SAT0361 Chest computed tomography as an alternative to high resolution manometry for the diagnosis of scleroderma esophagus. Ann Rheum Dis 2017; 76 (Suppl. 2) 908
    Search in Google Scholar
  • 21 Santos Duarte de Figueiredo AF, Ramalho M. Esophageal Dilatation as a Predictor of Systemic Sclerosis in Patients with Interstitial Lung Disease. Turk Thorac J 2021;
    CrossrefPubMedSearch in Google Scholar
  • 22 Keller J. Medikamentöse Therapie bei Motilitätsstörungen von Ösophagus und Magen. AVP 2016; 43: 4-14
    Search in Google Scholar
  • 23 Del Galdo F, Lescoat A, Conaghan PG. et al. EULAR recommendations for the treatment of systemic sclerosis: 2023 update. Ann Rheum Dis 2025; 84: 29-40
    CrossrefPubMedSearch in Google Scholar
  • 24 Snyder DL, Crowell MD, Kahn A. et al. Prevalence of Barrett’s Esophagus in Female Patients With Scleroderma. Am J Gastroenterol 2021; 116: 517-521
    CrossrefPubMedSearch in Google Scholar
  • 25 Foocharoen C, Chunlertrith K, Mairiang P. et al. Effectiveness of add-on therapy with domperidone vs. alginic acid in proton pump inhibitor partial response gastro-oesophageal reflux disease in systemic sclerosis: randomized placebo-controlled trial. Rheumatology 2017; 56: 214-222
    CrossrefPubMedSearch in Google Scholar
  • 26 Karamanolis GP, Panopoulos S, Karlaftis A. et al. Beneficial effect of the 5-HT1A receptor agonist buspirone on esophageal dysfunction associated with systemic sclerosis: A pilot study. United European Gastroenterol J 2015; 3: 266-271
    CrossrefPubMedSearch in Google Scholar
  • 27 Thonhofer R, Siegel C, Trummer M. et al. Early endoscopy in systemic sclerosis without gastrointestinal symptoms. Rheumatol Int 2012; 32: 165-168
    CrossrefPubMedSearch in Google Scholar
  • 28 Marie I, Gourcerol G, Leroi A. et al. Delayed gastric emptying determined using the 13C–octanoic acid breath test in patients with systemic sclerosis. Arthritis Rheum 2012; 64: 2346-2355
    CrossrefPubMedSearch in Google Scholar
  • 29 Camilleri M, Chedid V, Ford AC. et al. Gastroparesis. Nat Rev Dis Primers 2018; 4: 41
    CrossrefPubMedSearch in Google Scholar
  • 30 Keller J, Bassotti G, Clarke J. et al. Advances in the diagnosis and classification of gastric and intestinal motility disorders. Nat Rev Gastroenterol Hepatol 2018; 15: 291-308
    CrossrefPubMedSearch in Google Scholar
  • 31 Navas CM, Patel NK, Lacy BE. Gastroparesis: Medical and Therapeutic Advances. Dig Dis Sci 2017; 62: 2231-2240
    CrossrefPubMedSearch in Google Scholar
  • 32 Camilleri M, Atieh J. New Developments in Prokinetic Therapy for Gastric Motility Disorders. Front Pharmacol 2021; 12: 711500
    PubMedSearch in Google Scholar
  • 33 Ali H, Pamarthy R, Sarfraz S. Role of Prucalopride in Treating Functional Constipation and Gastroparesis: A Systemic Review. Cureus 2021; 13: e14306
    CrossrefPubMedSearch in Google Scholar
  • 34 Giudicessi JR, Ackerman MJ, Camilleri M. Cardiovascular safety of prokinetic agents: A focus on drug-induced arrhythmias. Neurogastroenterol Motil 2018; 30: e13302
    PubMedSearch in Google Scholar
  • 35 McMahan ZH, Volkmann ER. An update on the pharmacotherapeutic options and treatment strategies for systemic sclerosis. Expert Opin Pharmacother 2020; 21: 2041-2056
    PubMedSearch in Google Scholar
  • 36 Savarino E, Furnari M, de Bortoli N. et al. Gastrointestinal involvement in systemic sclerosis. Presse Med 2014; 43: e279-e291
    CrossrefPubMedSearch in Google Scholar
  • 37 Morrisroe K, Hansen D, Stevens W. et al. Gastric antral vascular ectasia in systemic sclerosis: a study of its epidemiology, disease characteristics and impact on survival. Arthritis Res Ther 2022; 24: 103
    CrossrefPubMedSearch in Google Scholar
  • 38 Parrado RH, Lemus HN, Coral-Alvarado PX. et al. Gastric Antral Vascular Ectasia in Systemic Sclerosis: Current Concepts. Int J Rheumatol 2015; 2015: 762546
    PubMedSearch in Google Scholar
  • 39 Peng M, Guo X, Yi F. et al. Endoscopic treatment for gastric antral vascular ectasia. Ther Adv Chronic Dis 2021; 12 20406223211039696
    CrossrefPubMedSearch in Google Scholar
  • 40 Efremova I, Maslennikov R, Poluektova E. et al. Epidemiology of small intestinal bacterial overgrowth. World J Gastroenterol 2023; 29: 3400-3421
    PubMedSearch in Google Scholar
  • 41 Valenzuela A, Li S, Becker L. et al. Intestinal pseudo-obstruction in patients with systemic sclerosis: An analysis of the Nationwide Inpatient Sample. Rheumatology (United Kingdom) 2016; 55: 654-658
    CrossrefPubMedSearch in Google Scholar
  • 42 Sakkas LI, Simopoulou T, Daoussis D. et al. Intestinal Involvement in Systemic Sclerosis: A Clinical Review. Dig Dis Sci 2018; 63: 834-844
    PubMedSearch in Google Scholar
  • 43 Kumar S, Singh J, Kedika R. et al. Role of muscarinic-3 receptor antibody in systemic sclerosis: correlation with disease duration and effects of IVIG. Am J Physiol Gastrointest Liver Physiol 2016; 310: G1052-G1060
    CrossrefPubMedSearch in Google Scholar
  • 44 Zandman-Goddard G, Tweezer-Zaks N, Shoenfeld Y. New therapeutic strategies for systemic sclerosis – A critical analysis of the literature. Clin Dev Immunol 2005; 12: 165-173
    PubMedSearch in Google Scholar
  • 45 Flanagan EP, Saito YA, Lennon VA. et al. Immunotherapy trial as diagnostic test in evaluating patients with presumed autoimmune gastrointestinal dysmotility. Neurogastroenterol Motil 2014; 26: 1285-1297
    CrossrefPubMedSearch in Google Scholar
  • 46 Raja J, Nihtyanova SI, Murray CD. et al. Sustained benefit from intravenous immunoglobulin therapy for gastrointestinal involvement in systemic sclerosis. Rheumatology (United Kingdom) 2016; 55: 115-119
    CrossrefPubMedSearch in Google Scholar
  • 47 Matsuda KM, Yoshizaki A, Kuzumi A. et al. Rapid improvement of systemic sclerosis-associated intestinal pseudo-obstruction with intravenous immunoglobulin administration. Rheumatology (United Kingdom) 2023; 62: 3139-3145
    CrossrefPubMedSearch in Google Scholar
  • 48 Soudah HC, Owyang C. Effect of octreotide on intestinal motility and bacterial overgrowth in scleroderma. N Engl J Med 1991; 325: 1461-1467
    PubMedSearch in Google Scholar
  • 49 Verne GN, Eaker EY, Hardy E. et al. Effect of octreotide and erythromycin on idiopathic and scleroderma-associated intestinal pseudoobstruction. Dig Dis Sci 1995; 40: 1892-1901
    PubMedSearch in Google Scholar
  • 50 Nikou GC, Toumpanakis C, Katsiari C. et al. Treatment of small intestinal disease in systemic sclerosis with octreotide: A prospective study in seven patients. J Clin Rheumatol 2007; 13: 119-123
    CrossrefPubMedSearch in Google Scholar
  • 51 Polkowska-Pruszyńska B, Gerkowicz A, Szczepanik-Kułak P. et al. Small intestinal bacterial overgrowth in systemic sclerosis: a review of the literature. Arch Dermatol Res 2019; 311: 1-8
    PubMedSearch in Google Scholar
  • 52 Pittman N, Rawn SM, Wang M. et al. Treatment of small intestinal bacterial overgrowth in systemic sclerosis: A systematic review. Rheumatology (United Kingdom) 2018; 57: 1802-1811
    Search in Google Scholar
  • 53 Ibáñez-Samaniego L, Baines García A, Alonso López S. Small intestinal bacterial overgrowth. Rev Esp Enferm Dig 2023; 115: 597-600
    CrossrefPubMedSearch in Google Scholar
  • 54 Shah A, Pakeerathan V, Jones MP. et al. Small Intestinal Bacterial Overgrowth Complicating Gastrointestinal Manifestations of Systemic Sclerosis: A Systematic Review and Meta-analysis. J Neurogastroenterol Motil 2023; 29: 132-144
    PubMedSearch in Google Scholar
  • 55 Morrisroe K, Baron M, Frech T. et al. Small intestinal bacterial overgrowth in systemic sclerosis. J Scleroderma Relat Disord 2020; 5: 33-39
    PubMedSearch in Google Scholar
  • 56 Parodi A, Sessarego M, Greco A. et al. Small intestinal bacterial overgrowth in patients suffering from scleroderma: Clinical effectiveness of its eradication. Am J Gastroenterol 2008; 103: 1257-1262
    CrossrefPubMedSearch in Google Scholar
  • 57 Ranjbar M, Naeini F, Rostamian A. et al. Effects of probiotics supplementation in gastrointestinal complications and quality of life of patients with systemic sclerosis: A systematic review. Heliyon 2024; 10: e36230
    PubMedSearch in Google Scholar
  • 58 Cheah JX, Perin J, Volkmann ER. et al. Slow Colonic Transit in Systemic Sclerosis: An Objective Assessment of Risk Factors and Clinical Phenotype. Arthritis Care Res (Hoboken) 2023; 75: 289-298
    CrossrefPubMedSearch in Google Scholar
  • 59 Hinton JM, Lennard-Jones JE, Young AC. A new method for studying gut transit times using radioopaque markers. Gut 1969; 10: 842-847
    CrossrefPubMedSearch in Google Scholar
  • 60 Vigone B, Caronni M, Severino A. et al. Preliminary safety and efficacy profile of prucalopride in the treatment of systemic sclerosis (SSc)-related intestinal involvement: Results from the open label cross-over PROGASS study. Arthritis Res Ther 2017; 19: 145
    CrossrefPubMedSearch in Google Scholar
  • 61 Sattar B, Chokshi RV. Colonic and Anorectal Manifestations of Systemic Sclerosis. Curr Gastroenterol Rep 2019; 21: 33
    PubMedSearch in Google Scholar
  • 62 Richard N, Hudson M, Gyger G. et al. Clinical correlates of faecal incontinence in systemic sclerosis: Identifying therapeutic avenues. Rheumatology (United Kingdom) 2017; 56: 581-588
    CrossrefPubMedSearch in Google Scholar
  • 63 Garros A, Marjoux S, Khouatra C. et al. Prevalence of fecal incontinence in a cohort of systemic sclerosis patients within a regional referral network. United European Gastroenterol J 2017; 5: 1046-1050
    CrossrefPubMedSearch in Google Scholar
  • 64 Thoua NM, Schizas A, Forbes A. et al. Internal anal sphincter atrophy in patients with systemic sclerosis. Rheumatology 2011; 50: 1596-1602
    CrossrefPubMedSearch in Google Scholar
  • 65 Jaffin BW, Chang P, Spiera H. Fecal Incontinence in Scleroderma. J Clin Gastroenterol 1997; 25: 513-517
    CrossrefPubMedSearch in Google Scholar
  • 66 Kenefick NJ, Vaizey CJ, Nicholls RJ. et al. Sacral nerve stimulation for faecal incontinence due to systemic sclerosis. Gut 2002; 51: 881-883
    PubMedSearch in Google Scholar
  • 67 Butt SK, Alam A, Cohen R. et al. Lack of effect of sacral nerve stimulation for incontinence in patients with systemic sclerosis. Colorectal Dis 2015; 17: 903-907
    CrossrefPubMedSearch in Google Scholar