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 Sjögren's syndrome. Four patients had scleroderma (all of them with Sjörgren's syndrome), nine had Sjögren's syndrome without scleroderma, and five had neither scleroderma nor Sjögren's syndrome. Three patients with scleroderma had aperistalsis and diminished lower sphincter pressure. Five patients with Sjörgren'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 Sjögren'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 Sjögren'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 Sjögren's syndrome without scleroderma, suggesting that some esophageal motor disturbances could be related to association with Sjögren's syndrome.