Esophageal dysfunction in primary biliary cirrhosis

To investigate esophageal involvement of scleroderma in primary biliary cirrhosis, esophageal, manometry was performed in 18 patients (16 females, two males) with primary biliary cirrhosis and in a control group of 18 subjects matched by age and sex. All patients were screened for clinical manifestations of scleroderma and for the presence of Sj?gren's syndrome. Four patients had scleroderma (all of them with Sj?rgren's syndrome), nine had Sj?gren's syndrome without scleroderma, and five had neither scleroderma nor Sj?gren's syndrome. Three patients with scleroderma had aperistalsis and diminished lower sphincter pressure. Five patients with Sj?rgren's syndrome without scleroderma also had esophageal manometric disturbances. Furthermore, lower esophageal sphincter pressure (LESP) and distal mean wave pressure (DMWP) were significantly reduced in patients with scleroderma (LESP: 7.5 +/- 1.4 mmHg; DMWP: 29.5 +/- 5.9 mmHg) and in patients with Sj?gren's syndrome without scleroderma (LESP: 14.8 +/- 0.8 mmHg; DMWP: 54.3 +/- 7.5 mmHg) compared to controls (LESP: 18.0 +/- 0.7 mmHg; DMWP: 83.9 +/- 5.1 mmHg). By contrast, LESP and DMWP were similar in patients without Sj?gren's syndrome (LESP: 17.6 +/- 0.9 mmHg; DMWP: 78.2 +/- 10.9 mmHg) and controls. These results indicate that esophageal motility dysfunction is often present in patients with primary biliary cirrhosis who have scleroderma, and also in those with Sj?gren's syndrome without scleroderma, suggesting that some esophageal motor disturbances could be related to association with Sj?gren's syndrome.