The association between systemic sclerosis disease manifestations and esophageal high-resolution manometry parameters

Abstract

Background

We aimed to evaluate the associations between systemic sclerosis (SSc)-related systemic manifestations and esophageal function using high-resolution manometry (HRM).

Methods

Patients with SSc that had undergone HRM between 1/2004 and 9/2014 were identified and HRMs were analyzed according to the Chicago Classification. Clinical characteristics were identified via retrospective chart review and compared among motility diagnoses while adjusting for age, gender, race, and SSc-disease duration.

Key Results

Seventy-nine patients (85% female, ages 25–77) were included. Clinical characteristics were compared between patients with absent contractility (AC, n = 40), ineffective esophageal motility (IEM; n = 15), and normal motility (n = 19); the five remaining patients met criteria for other motility diagnoses. Groups differed in severity of skin involvement measured by the modified Rodnan skin score (0–51): AC (adjusted mean 12.6), IEM (4.4), normal (4.3), p = 0.043. Pulmonary function tests [percent predicted FVC and DLCO) were lower in AC (adjusted mean, FVC: 70.3, DLCO 51.1), than IEM (FVC: 92.0; DLCO: 76.9) and normal motility (FVC: 80.0; DLCO: 67.2), p values 0.057 (FVC) and 0.007 (DLCO). Groups did not differ by SSc-disease duration, autoantibodies, or reported symptoms of dysphagia or reflux.

Conclusions & Inferences

In patients with SSc, absent esophageal contractility on HRM was associated with increased skin disease severity and worse lung function. Obtaining HRM to identify SSc patients with more severe esophageal dysfunction could be considered to enable implementation of management strategies in patients potentially at risk for increased morbidity and mortality.

Patients with systemic sclerosis (SSc) and previous completed high-resolution manometry were retrospectively evaluated to assign an esophageal motility diagnosis by the Chicago Classification and explore associations with SSc-related clinical characteristics. We identified an association of increased skin severity (measured by the Modified Rodnan Skin Score) and more severe reductions in pulmonary function tests in patients with absent contractility compared with patients with ineffective esophageal motility and normal esophageal motility.

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