Background/Purpose: Pathogenic mechanisms of esophageal involvement in systemic sclerosis (SSc) were suggested as neural dysfunction due to impairment of microcirculation to intramural neuron in early stage, followed by atrophy of smooth muscle (SM) portion, and finally extensive fibrosis. According to the esophageal high resolution manometry (HRM) finding, three are three categories in SSc patients; normal peristalsis, ineffective esophageal motility (IEM), and absent contractility (AC). Our hypothesis is IEM of HRM finding in SSc patients may reflect neural dysfunction with/without SM atrophy and AC may reflect severe SM atrophy or extensive fibrosis. Aims of our study are to evaluate the esophageal reserved function of neuronal reflexes and muscle contractility in these three groups of SSc.

Methods: Patients diagnosed with SSc who underwent HRM during recent 3 years were retrospectively included (22 female, ages 25-75). Seventeen patients of them were underwent multichannel intraluminal impedance and pH (Imp-pH) study also. We analyzed LES pressure, HRM metrics according to the Chicago classification version 3, including distal contractile integral (DCI), DCI ratio of single swallow and multiple rapid swallow (MRS) test, and reflux parameters and the post-reflux swallow-induced peristaltic wave (PSPW) index by Imp-pH test. According to the HRM findings, patients were classified as 3 groups (normal HRM, n=5; IEM, n=8, and AC, n=9). We compared SM contractility by DCI, neuronal peristaltic reserve function by MRS test using HRM and the PSPW index using Imp-pH study in normal, IEM, and AC groups. Parameters among 3 groups were analyzed by ANOVA on ranks.

Results : DCI was significantly lower in AC and IEM group than normal group [median 0.00 (IQR 0.00-35.23), 172.80 (77.05-522.35), and 834.30 (789.85-2294.63) mmHg×s×cm respectively, p<0.001]. DCI ratio of MRS/single swallow was lower in AC group than normal group [0.00 (0.00-0.08) vs. 1.00 (0.45-1.19), P=0.030]. Other HRM parameters including LES pressure were not different among three groups. PSPW index was lower in AC group than IEM and normal groups [0.40 (0.00-1.90), 8.95 (3.20-16.70), and 17.60 (10.0-35.40) % respectively, p=0.023]. PSPW appearance time until 120 seconds were tended to variable in IEM group and AC group than normal group [SD 34.28 (32.29-44.89), 27.60 (16.97-32.03) and 19.60 (17.95-28.02) seconds respectively, p=0.079]. Other reflux parameters were not different among 3 groups.

Conclusion: The AC using HRM may reflect all of SM atrophy, fibrosis and neuronal dysfunction and the IEM using HRM may reflect SM atrophy and mild to moderate neuronal dysfunction in patients with SSc (Figure 1).

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pathogenic-mechanisms-of-esophageal-peristaltic-dysfunction-by-high-resolution-manometry-in-patients-with-systemic-sclerosis/