Progression of esophageal dysmotility in systemic sclerosis

To longitudinally evaluate esophageal dysmotility (ED) in patients with limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc).

We performed a retrospective review of all adult patients with SSc seen between 1995 and 2008. Patients were included if they had undergone 2 or more esophageal transit scintigraphy (ETS) studies at least 1 year apart. Data from 382 ETS studies of 102 patients with SSc were analyzed. Eighty patients had lcSSc and 22 patients had dcSSc. A grading system was used to quantify the degree of esophageal dysfunction, ranging from grade 0 (normal) to grade 3 (severe hypomotility). Change in esophageal motility over time was evaluated and compared between the limited and diffuse subtypes.

Sixty-eight patients (66.7%) had an abnormal ETS study at any time. Of patients with dcSSc, 95.4% had an abnormal ETS study, compared to 58.5% of patients with lcSSc. dcSSc and regurgitation were independent risk factors for ED. There was no association between the presence of anticentromere antibodies or antitopoisomerase (anti-Scl-70) antibodies and an abnormal ETS study. Esophageal motility in patients with dcSSc worsened in 96% of cases compared with only 58.8% in those with lcSSc.

ED is more frequent in patients with dcSSc than in those with lcSSc, and is more likely to deteriorate over time. Given the potential associated risks of erosive esophagitis, Barrett's esophagus, and esophageal cancer in patients with SSc, routine screening and monitoring for ED is advised.