Esophageal dysmotility associated with systemic sclerosis: a high-resolution manometry study

Abstract

Esophageal involvement occurs in about 80% of patients with systemic sclerosis, with a marked diminution of peristaltic pressures in the distal two-thirds of the esophagus. Our aims were to more fully characterize esophageal motility disorders in systemic sclerosis using high-resolution manometry (HRM) and to determine predictive factors of esophageal involvement. Fifty-one patients (46 females) with systemic sclerosis were included in this retrospective study. Esophageal motility was characterized with HRM. The demographic data, esophageal symptoms, presence of other organ involvement, and autoantibody profile (anti-Scl70 antibodies [Scl70], anticentromere antibodies [ACA]) were recorded for all patients. Esophageal body dysmotility was present in 33 patients (67.3%) and was associated with hypotensive esophagogastric junction in 27 patients (55.1%). The velocity of proximal contractions was higher in patients with esophageal body dysmotility compared to patients with normal peristalsis (median 10.8 cm/s vs. 5.5, P = 0.04). The amplitude of middle esophageal contraction but not of distal esophageal contraction was reduced in patients with hypoperistalsis. Diffuse esophageal skin involvement, presence of Scl70 and absence of ACA were associated with esophageal involvement. Esophageal symptoms encountered in 87.5% of patients were not predictive of esophageal dysmotility. This HRM series confirms the high prevalence of esophageal body dysmotility in systemic sclerosis. Diffuse skin involvement, positive Scl70 and negative ACA, but not esophageal symptoms, may predict esophageal body dysmotility.

Loss of Peristaltic Reserve, Determined by Multiple Rapid Swallows, Is the Most Frequent Esophageal Motility Abnormality in Patients With Systemic Sclerosis

“…11-13 Another study using HRM to evaluate patients with SSc reported similar frequencies of esophageal motor patterns as our current study: 22/48 (46%) had absent peristalsis, 10/48 (21%) had hypotensive peristalsis and 16/48 (33%) had normal peristalsis. 12 …”

Management of Gastrointestinal Involvement in Scleroderma

“…The esophagus is involved early in SSc and is the most frequently involved portion of the GIT affecting approximately 80% of patients [6, 11]. While the majority of patients are symptomatic, the absence of symptoms does not exclude esophageal dysfunction [11-13].…”

“…While the majority of patients are symptomatic, the absence of symptoms does not exclude esophageal dysfunction [11-13]. …”

Advances in the Evaluation and Management of Esophageal Disease of Systemic Sclerosis

“…Despite its prevalence, the understanding of the pathogenesis of esophageal dysfunction in SSc remains relatively poor; mechanisms involving vascular injury and ischemia, neurodegeneration, and collagen deposition causing muscular atrophy and fibrosis are considered [3–5]. Various SSc clinical manifestations including disease subtype, serum autoantibodies, skin findings, and Raynaud’s phenomenon have been found to be associated with increased prevalence of SSc esophageal disease [6–8]. The most notable association, however, is interstitial lung disease which is the primary cause of mortality in SSc [9–14].…”

“…Symptoms are poorly correlated with objective findings of esophageal disease in patients with SSc [7, 8]. Common abnormalities in gastrointestinal function include weak or absent distal esophageal peristalsis and hypotensive lower esophageal sphincter (LES) pressure, a pattern often termed scleroderma esophagus (even in the absence of SSc).…”