Systemic sclerosis & Raynaud's

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

Rheumatology (Oxford)Published Cohort study

Authors' conclusion

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.

Study context

Population
Patients with SSc with detailed clinical and immunological data (84% female, mean age 45 ± 17 years)442 person-years follow-up
Participants
201 patients
Exposure
Oesophageal motility patterns by high-resolution oesophageal manometry: Absent contractility (AC) vs IEM vs normal motility
  • ACn = 86
  • IEMn = 57
  • Normal motilityn = 58
Outcome
Adverse outcomes:
  • Death
  • Lung transplantation
Methodological detailsKaplan-Meier and Cox regression analyses, Chicago 4.0 classification
  • Kaplan-Meier and Cox regression analyses
  • Chicago 4.0 classification

Main finding

As reported in the abstract

ACHazard ratio (95% CI)P value
Lung transplantation; adjusted for ILD and male sex
7.004(1.481–33.135)
P = 0.014
SSc-related death; adjusted for dcSSc and ILD
3.472(1.071–10.969)
P = 0.038

Observational data: associations, not effects.

Further findings

Clinical phenotypes by motility pattern

AC associated with dcSSc, more severe digital ulcers, gastric vascular ectasia, anti-Ro60 antibodies and a late nailfold capillaroscopy pattern
P < 0.05
IEM linked to limited SSc, anti-centromere antibodies and an early/active nailfold pattern

Original publication

Oesophageal dysmotility patterns are associated with distinct clinical phenotypes and prognosis in patients with systemic sclerosis. Rheumatology (Oxford). Published 9 August 2025. doi:10.1093/rheumatology/keaf433

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