Introduction
Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by vasculopathy and fibrosis. The gastrointestinal tract is one of the most frequently involved systems, manifesting as hypomotility of the affected segments. As SSc survival rates increase, digestive manifestations are becoming more important for the comprehensive management of affected patients.
Aims & Methods
Objective: To analyze the prevalence of digestive symptoms and dysmotility of the gastrointestinal tract in patients with systemic sclerosis and to assess their impact on the quality of life.
Methods: Cross-sectional study in a cohort of active patients with SSc, performed between January and July 2022. Digestive symptoms were evaluated by standardized questionnaires (UCLA-GIT 2.0 and a motility symptoms questionnaire). Health-related QoL was evaluated using the SF-36 questionnaire. Demographic, clinical-immunological data and results of examinations (upper endoscopy, esophageal manometry, gastric emptying scintigraphy, intestinal manometry, and CT scans) were collected from clinical records.
Results
183 patients with SSc participated in the study (age 59±12 years, 103 (55%) patients had limited cutaneous SSc, 43 (24%) diffuse SSc, and 37 (21%) sine-sclerosis SSc). 139 (77%) patients reported having at least one moderate digestive symptom (>1 day/week). The prevalence of digestive symptoms correlated negatively with overall quality of life measured by SF-36 (r= -0.503, IC -0.615 to -0.397, p=<0.001). Gastrointestinal motility assessments detected esophageal motor abnormalities in 67/123 (55%) patients, gastroparesis in 18/31 (58%) patients (mild n=6, moderate n=5, severe n=7), small bowel dysmotility by manometry in 6/6 (100%) (neuropathic n=1, myopathic n=5), and dilated small bowel loops by CT scan in 6/65 (9.2%) patients. Severe gastrointestinal involvement (gastroparesis and/or small bowel dysmotility, n=21, 11%), was associated with older age at SSc onset (45 ± 9 vs. 36 ± 14 years; p=0.012), the diffuse subtype (57% vs 18%; p=0.001), myositis (24% vs. 3%, p=0.011), tendon friction rubs (29% vs 3%, p=0.003) and with erosive esophagitis (10% vs. 57%, p=0.001). Patients with severe gastrointestinal involvement had a higher digestive burden, measured by UCLA GIT 2.0 score (1.05 ± 0.63 vs 0.62 ± 0.51; p=0.043) mainly due to reflux subscore (1.07 ± 0.63 vs 0.58 ± 0.61; p=0.001) and bloating subscore (1.85 ± 0.78 vs 1.23 ± 0.94; p=0.001)
Conclusion
Patients with SSc present a high prevalence of digestive symptoms and objective gastrointestinal dysmotility that negatively influence their quality of life. SSC patients with severe gastrointestinal dysmotility may represent a different disease phenotype.
References
Denton CP, De Lorenzis E, Roblin E, Goldman N, Alcacer-Pitarch B, Blamont E, Buch M, Carulli M, Cotton C, Del Galdo F, Derrett-Smith E, Douglas K, Farrington S, Fligelstone K, Gompels L, Griffiths B, Herrick A, Hughes M, Pain C, Pantano G, Pauling J, Prabu A, O'Donoghue N, Renzoni E, Royle J, Samaranayaka M, Spierings J, Tynan A, Warburton L, Ong V. Management of systemic sclerosis: British Society for Rheumatology guideline scope. Rheumatol Adv Pract. 2023 Mar 14;7(1):rkad022. doi: 10.1093/rap/rkad022.
McMahan ZH, Kulkarni S, Chen J, Chen JZ, Xavier RJ, Pasricha PJ, Khanna D. Systemic sclerosis gastrointestinal dysmotility: risk factors, pathophysiology, diagnosis and management. Nat Rev Rheumatol. 2023 Mar;19(3):166-181. doi: 10.1038/s41584-022-00900-6.
Disclosure
Nothing to disclouse
