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

Abstract

Background/Aims We aimed to evaluate the associations between 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. Results 79 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 5 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 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.

Abnormal esophageal motility during a solid test meal in systemic sclerosis—detection even in very early disease and association with disease progression

“…Consistent with the results of the majority of studies, the frequency and intensity of GI symptoms reported on questionnaires did not associate with SSc disease stage in our patient cohort (Table ). The discrepancy between questionnaire data and pathological findings on HRM testing might be explained by reduced esophageal sensitivity, although the results of previous studies yielded mixed results .…”

“…In recent years, classification of SSc has evolved and disease groups in previous studies are not necessarily comparable to current investigations. To date, three HRM studies have documented the association of esophageal motility assessed by SWS with SSc disease activity . A small prospective study from our group focused on the treatment of reflux disease in 14 SSc patients, and two recent clinical series showed a link between esophageal motility and lung disease.…”

The Effect of Anti-reflux Therapy on Patients Diagnosed with Minor Disorders of Peristalsis in High-resolution Manometry

“…[4][5][6] Other studies have suggested that IEM is closely associated with systematic diseases, including systemic sclerosis and chagas disease. [7][8][9] However, a recent study reported that there was no significant relationship between minor peristaltic disorders and symptoms. Moreover, minor disorders of peristalsis are unaffected by the management.…”

A randomized double‐blind placebo‐controlled crossover‐style trial of buspirone in functional dysphagia and ineffective esophageal motility

“…According to the Chicago Classification v3.0, IEM is a manometric diagnosis in which the Integrated Residual Pressure (IRP) is less than 15 mm Hg, but ≥50% of swallows are either weak or failed (Distal Contractile Index [DCI] is ≤450 mm Hg) . IEM has been shown to be a possible pathophysiologic mechanism in patients with otherwise unexplained dysphagia and various series report an increase in the prevalence of IEM in patients with systemic sclerosis and Parkinson's disease …”