BACKGROUND AND AIMS Study of health related quality of life (HRQOL) and the factors responsible for its impairment in primary biliary cirrhosis (PBC) has, to date, been limited. There is increasing need for a HRQOL questionnaire which is specific to PBC. The aim of this study was to develop, validate, and evaluate a patient based PBC specific HRQOL measure. SUBJECTS AND METHODS A pool of pote...

Fatigue is probably the most intriguing symptom affecting patients with chronic cholestatic disorders, in particular those with primary biliary cirrhosis. It is postulated that fatigue in patients with primary biliary cirrhosis may be associated with morphological abnormalities of the central nervous system secondary to accumulation of manganese. However, we are still far from understanding thi...

INTRODUCTION We report an unusual presentation of primary biliary cirrhosis. CASE PRESENTATION We present the case of a 66-year-old white British woman who presented to the Accident and Emergency department with ascites and unilateral pleural effusion (hepatic hydrothorax). Following a liver biopsy, the diagnosis of primary biliary cirrhosis was made. CONCLUSION It is important to consider ...

The association of finger clubbing and periostitis has been reported in primary biliary cirrhosis and, more rarely, in other forms of chronic liver disease. The prevalence of periostitis and its relationship to finger clubbing is unknown. In this prospective study, we have determined the prevalence of periostitis and finger clubbing in 74 patients with primary biliary cirrhosis and 54 with othe...

Antimitochondrial antibodies are found in 85 to 95% of patients with primary biliary cirrhosis. Nine different patterns have been identified but only four are associated with primary biliary cirrhosis. Anti-M2 antibodies are specific for the disease. The M2 antigen was found to be composed of five antigen determinants and related to the E2 component of three multienzyme complexes located within...

Two West Indian patients with Kveim-biopsy proven sarcoidosis developed chronic cholestatic liver disease, clinically and biochemically similar to primary biliary cirrhosis. Liver histology revealed multiple granulomas with reduction in bile ducts and, in one patient, progression to biliary cirrhosis. Portal hypertension was present in both patients leading to severe variceal haemorrhage in one...

A case is described of primary biliary cirrhosis in a 59 year old woman who initially presented with a rash over her lower legs 18 months before diagnosis. Skin biopsy examination showed non-caseating granulomas of the sarcoid type. It is believed that this is the first reported case of primary biliary cirrhosis presenting with a granulomatous rash.

Mallory's (;alcoholic') hyaline has been found in hepatocytes in 18 of 70 patients with primary biliary cirrhosis. These inclusions have previously been noted in only three cases of primary biliary cirrhosis. Current views on the nature of Mallory's hyaline are briefly discussed.

Primary Biliary Cirrhosis and Myasthenia Gravis are both autoimmune conditions, however, there are only rare case reports of their association. This is a case report of acetylcholine receptor antibody positive generalized myasthenia gravis in a female patient with antimitochondrial antibody positive, liver biopsy-confirmed primary biliary cirrhosis.