Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2025 Dec 1;64(12):6250-6258.
doi: 10.1093/rheumatology/keaf433.

Oesophageal dysmotility patterns are associated with distinct clinical phenotypes and prognosis in patients with systemic sclerosis

Luis G Alcala-Gonzalez  1 , Alfredo Guillen-Del-Castillo  2 , Ariadna Aguilar  1   3 , Claudia Barber  1   3 , Carolina Malagelada  1   3   4 , Laura Polo Figueras  1 , Laura Triginer  2 , Claudia Codina-Clavaguera  2 , Michael Hughes  5   6 , Jordi Serra  1   3 , Carmen P Simeón-Aznar  2 , Zsuzsanna H McMahan  7
Affiliations

Oesophageal dysmotility patterns are associated with distinct clinical phenotypes and prognosis in patients with systemic sclerosis

Luis G Alcala-Gonzalez et al. Rheumatology (Oxford). .

Abstract

Objectives: Oesophageal dysmotility is a common manifestation of SSc, contributing to substantial morbidity. We sought to determine whether oesophageal dysmotility patterns by high-resolution oesophageal manometry were associated with distinct SSc clinical phenotypes and different outcomes.

Methods: We analysed a cohort of SSc patients with detailed clinical and immunological data. Oesophageal motility was classified using Chicago 4.0 criteria, and baseline characteristics were compared across motility patterns [absent contractility (AC), ineffective oesophageal motility (IEM) and normal motility]. Associations with adverse outcomes (death or lung transplantation) were evaluated using Kaplan-Meier and Cox regression analyses.

Results: Our cohort included 201 patients with SSc (84% female, mean age 45 ± 17 years, follow-up 442 person-years). Oesophageal dysmotility patterns were classified as AC in 86 (43%), IEM in 57 (28%) and normal motility in 58 (29%). AC was associated with dcSSc, more severe digital ulcers, gastric vascular ectasia, anti-Ro60 antibodies and a late pattern on nailfold capillaroscopy (P < 0.05), while IEM was linked to limited SSc, anti-centromere antibodies and an early/active nailfold pattern. Multivariate time-to-event analysis identified AC as an independent risk factor for lung transplantation (HR = 7.004, 95%CI: 1.481-33.135, P = 0.014) after adjusting for both interstitial lung disease and male sex, and for SSc-related death (HR = 3.472, 95%CI: 1.071-10.969, P = 0.038) after adjusting for DcSSc and interstitial lung disease.

Conclusions: We found that patients with AC and IEM have distinct clinical phenotypes, suggesting they are distinct entities. In patients with SSc, AC is independently associated with worse outcomes. These data suggest that HREM may be useful in risk stratification and outcome predictions in patients with SSc.

Keywords: gastrointestinal involvement; high-resolution manometry; oesophageal dysmotility; systemic sclerosis.

PubMed Disclaimer

References

    1. McMahan ZH, Kulkarni S, Chen J et al. Systemic sclerosis gastrointestinal dysmotility: risk factors, pathophysiology, diagnosis and management. Nat Rev Rheumatol 2023;19:166–81. - PubMed
    1. Bandini G, Alunno A, Ruaro B et al. Significant gastrointestinal unmet needs in patients with Systemic Sclerosis: insights from a large international patient survey. Rheumatology 2024;63:e92–e93. - PMC - PubMed
    1. Bandini G, Alunno A, Pinheiro FO et al. A multinational survey investigating the unmet needs and patient perspectives concerning proton pump inhibitors in systemic sclerosis. Arthritis Care Res 2024;76:608–15. - PubMed
    1. Alcala-Gonzalez LG, Guillen-Del-Castillo A, Simeón-Aznar CP; Scleroderma GI Research Working Group, Annex. Scleroderma GI Research Working Group. Patient perspectives and treatment gaps in managing gastrointestinal symptoms in systemic sclerosis. Med Clin (Barc) 2025;164:196–8. - PubMed
    1. Kaniecki T, Abdi T, McMahan ZH. A practical approach to the evaluation and management of gastrointestinal symptoms in patients with systemic sclerosis. Best Pract Res Clin Rheumatol 2021;35:101666. - PMC - PubMed