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Background: It is common for patients with Systemic Sclerosis (SSc) to experience gastrointestinal (GI) symptoms at some point during the disease, with up to 90% of patients affected 1. The clinical expression of GI involvement can vary widely, and pathogenesis is not entirely understood. It is known that the autonomic nervous system plays a crucial role in controlling GI motility 2, and the dysfunction of the sympathetic and parasympathetic pathways could contribute to the clinical heterogeneity in SSc disease 3. Objectives: To identify patients with systemic sclerosis (SSc) who have a high burden of autonomic symptoms and to determine whether they have a distinct clinical phenotype and specific pattern of gastrointestinal (GI) transit. Methods: In a prospective cohort of SSc patients with GI disease, clinical data were obtained at routine clinical visits. Autonomic symptoms were identified using the validated Composite Autonomic Symptom Score, COMPASS-31 questionnaire. GI transit was measured by whole-gut scintigraphy. Clinical, demographic, and GI transit characteristics were compared between patients with global autonomic dysfunction (GAD); ≥5 positive COMPASS-31 subdomains and those with limited autonomic dysfunction (LAD); Results: 88 patients with SSc and GI involvement were included. The mean age was 57 years (IQR 48-65), 90% were female, and 74% had limited cutaneous disease. 82% had significant GI disease (Medsger score ≥2), and 72% had sicca syndrome. The mean COMPASS-31 score was 36.7 ± 14.2 (SD) (Table 1), 31% had GAD and 69% had LAD. Patients with GAD were more likely to be women (100% vs. 85%; p=0.052), have sicca symptoms (100% vs. 83%; p=0.027), upper GI involvement (Medsger GI score of 1 or 2) (96% vs. 78%; p=0.03) (Figure 1), and faster gastric emptying of solids at 4 hours (97% vs. 94% p =0.023). Conclusion: Our study highlights the significance of the autonomic nervous system in SSc patients with GI involvement. We have identified the characteristics of a specific group of patients affected by significant symptoms of dysautonomia and examined the relationship between autonomic symptom burden and GI motility. Our findings may pave the way for developing novel treatments that target the autonomic nervous system and improve the quality of life of these patients. REFERENCES: [1 Volkmann ER, McMahan Z. Gastrointestinal involvement in systemic sclerosis: pathogenesis, assessment and treatment. Vol. 34, Curr Opin Rheumatol. Lippincott Williams and Wilkins; 2022. p. 328–36. 2 Robert W. Sjogren. Gastrointestinal features of scleroderma. Curr Opin Rheumatol.1996 Nov;8(6):569-75. 3 Da Silva H, Pardaens S, Vanderheyden M, De Sutter J, Demeyer H, De Pauw M, Demulier L, Stautemas J, Calders P. Autonomic symptoms and associated factors in patients with chronic heart failure. Acta Cardiol. 2023 Apr;78(2):203-211. doi: 10.1080/00015385.2021.2010953. Epub 2021 Dec 9. PMID: 3488675. Acknowledgements: We would like to acknowledge Michelle Leatherman and Adrianne Woods for their support. Disclosure of Interests: María Paula Álvarez Hernandez: None declared, Brittany Adler: None declared, Jamie Perin: None declared, Michael Hughes Speaking fees from Actelion, Eli Lilly, Janssen and Pfizer, outside the submitted work., Research funding from Janssen,outside of the submitted work. Member of a data and safety monitoring board for Certa Therapeutics. Chair of a data and safety monitoring board for a digital ulcer trial (NIHR funded)., Zsuzsanna McMahan: None declared.
Hernández et al. (Sat,) studied this question.