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

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.