Sjögren's syndrome in patients with primary biliary cirrhosis

Abstract

Symptomatology and objective findings of Sjögren's syndrome were evaluated in 38 consecutive patients with primary biliary cirrhosis. Symptoms of Sjögren's syndrome were present in 18 (47.4%) patients, but were severe enough to warrant therapy in only four (10.5%). Nineteen patients consented to evaluation for Sjögren's syndrome, which included Schirmer's I test, measurement of parotid flow rate and serum autoantibodies, labial minor salivary gland biopsy and human leukocyte antigen typing. Histological changes diagnostic of Sjögren's syndrome were present in five patients (26.3%). All five patients had symptoms of Sjögren's syndrome and three had abnormal Schirmer's I tests, but none had corneal ulcerations or decreased parotid flow rates. Results of serological tests and human leukocyte antigen typing were not similar to those described in patients with primary Sjögren's syndrome but were similar to those described in patients with rheumatoid arthritis and Sjögren's syndrome. These findings indicate that Sjögren's syndrome associated with primary biliary cirrhosis is a form of secondary Sjögren's syndrome resembling that associated with rheumatoid arthritis.

Decreased anion exchanger 2 immunoreactivity in the liver of patients with primary biliary cirrhosis

“…15,16 We hypothesized that abnormalities in bicarbonscores were observed among control groups. When four ate transport mechanisms could underlie both the cholestasis patients with primary sclerosing cholangitis (PSC) were and the exocrine failure that characterize this disease.…”

“…13,14 group (normal liver and cirrhosis or cholestasis other PBC is frequently associated with pluriglandular exocrine than PBC), whereas no differences in immunoreactivity failure. 15,16 We hypothesized that abnormalities in bicarbonscores were observed among control groups. When four ate transport mechanisms could underlie both the cholestasis patients with primary sclerosing cholangitis (PSC) were and the exocrine failure that characterize this disease.…”

Evidence for a locally driven mucosal response and the presence of mitochondrial antigens in saliva in primary biliary cirrhosis

“…The fact that PBC has been associated with a number of other autoimmune diseases in which tissues of the secretory system are involved, such as rheumatoid arthritis, Sjogren' s syndrome, chronic thyroiditis, and CREST syndrome, 7,8 has led to studies of secretory tissues from PBC patients. The results of these studies concluded that although the principal target of PBC is the intrahepatic small bile ducts of the liver, pathology was also noted within ductal epithelial cells in the pancreas, salivary, and lacrimal glands.…”

Mucosal Immunity and Primary Biliary Cirrhosis: Presence of Antimitochondrial Antibodies in Urine

“…1,2 Furthermore, the concept of dry gland syndrome is used to explain the frequent occurrence of dry eyes or dry mouth in patients with PBC, 3 and there are certain similarities between Sjö gren's syndrome and PBC. 4,5 Our laboratory has generated a series of murine monoclonal and human combinatorial antibodies against PDC-E2, the major mitochondrial autoantigen. Use of these monoclonal antibodies led to the finding that some of these antibodies produce an abnormal apical staining of biliary epithelial cells only in liver tissues from PBC but not controls or patients with other liver diseases.…”