Primary biliary cirrhosis

Primary biliary cirrhosis often presents insidiously but progresses relentlessly in its later stages. Asymptomatic patients, however, may have a relatively benign course. Clinical, laboratory and histologic characteristics support an autoimmune pathogenesis. The complications of primary biliary cirrhosis are secondary to severe cholestasis, portal hypertension and progressive hepatocellular dysfunction. Currently, treatment is only supportive; no drug regimen has been shown to alter the disease course.     

Primary biliary cirrhosis

Primary biliary cirrhosis is a chronic liver disease of unknown etiology characterized by slowly progressive intrahepatic cholestasis due to an inflammatory destruction of small intrahepatic bile ducts. The clinical course of PBC is variable ranging from a few years in rapidly progressive cases to a normal life-expectancy in a proportion of asymptomatic cases. The typical patient is a middle-aged woman who may present with pruritus, increasing pigmentation of the skin, and eventually jaundice. The level of serum alkaline phosphatase is almost invariably elevated, serum mitochondrial antibodies are present in more than 90 per cent, and an elevated serum IgM is usually present. PBC is associated with many immunologic abnormalities and appears to be a classic autoimmune disease. Some of the immune defects may be epiphenomena; others such as a marked defect in suppressor T cell function seem to be related to the pathogenesis of the disease. All drug therapy that is aimed at slowing the disease process is experimental. A place for immunosuppressive drugs in the management of PBC would be anticipated. However, no drug has to date been definitively shown to have a beneficial effect on the disease. Currently, the main treatments used are aimed at preventing or correcting the complications of intractable cholestasis. Patients with PBC and evidence of hepatic decompensation and/or poor quality of life make good candidates for liver transplantation. The current aim of therapy is to find an effective regime of immunosuppression that will make hepatic transplantation redundant for this disease.     

Gout gone awry: The importance of proper diagnosis

Gout is the best‐known type of arthritis with a prevalence of 1%–3% in the western world (Therapeutic Advances in Musculoskeletal Disease, 6, 2014 and 131; Journal of Advanced Research, 8, 2017 and 495). Although it is well understood, there is growing evidence of the misdiagnosis of gout from other forms of arthritis. These errors lead to delay in accurate diagnosis (Journal of Advanced Research, 8, 2017 and 495).     

Primary biliary cirrhosis: current diagnosis and treatment.

Primary biliary cirrhosis is a progressive noninflammatory destruction of the interlobular bile ducts within the liver, leading to cholestasis and eventual cirrhosis. Ninety percent of affected patients are women. Most patients are initially without symptoms or have mild symptoms such as fatigue or pruritus. A minority of patients have the classical triad of jaundice, pruritus, and xanthelasmas. Almost all patients will have positive anti-mitochondrial antibody test results and an elevation of the serum alkaline phosphatase level. Primary biliary cirrhosis is thought to be an autoimmune disorder with additional liver injury being mediated by the subsequent cholestasis and accumulation of toxic bile acids. New treatment modalities include colchicine, ursodeoxycholic acid, and methotrexate. All patients, including those with only minor symptoms, have increased mortality compared with age-matched controls, thereby emphasizing the need for early diagnosis.     

Primary biliary cirrhosis accompanied by CREST syndrome

CREST syndrome, a relatively benign, slowly progressive variant of systemic scleroderma consists of calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. Although the association of this syndrome with primary biliary cirrhosis (PBC) is recognized in women, it has never been described in a man. We report the rare case of a male patient with CREST syndrome accompanied by PBC, manifested by acute cholecystitis and mild jaundice. The association of the two conditions is clinically and etiologically important. Clinicians must be aware of this association, sincethe clinical features of CREST syndrome may be mild and may be thought to be complications of the underlying liver disease.     

原发性胆汁性肝硬化18例临床分析

原发性胆汁性肝硬化(PBC)是一种慢性肝内胆汁淤积性疾病,以往认为我国少见,随着人们对此病认识和医学免疫学检测技术的提高,对PBC的临床表现及诊断有了进一步认识,使PBC患者得到早期诊断和治疗。1998年1月～2002年5月我院经肝穿刺及尸检病理学等检查确诊PBC 18例,现分析临床特点如下。     

Primary biliary cirrhosis: 12-year experience in observation

AIM: To ascertain initial signs of primary biliary cirrhosis (PBC), criteria of its early diagnosis, variants of the course, clinical picture and life quality of PBC patients. MATERIAL AND METHODS: 128 PBC patients were examined in the Central Research Institute of Gastroenterology from 1991 to 2002. Quality of life was assessed by SF-36 questionnaire adapted for patients with chronic cholestatic diseases of the liver. RESULTS: The patients complained of skin puritus (80%), jaundice (66%), fatigue (68.8%), weight loss (24.6%). The disease manifested with skin pruritis in 75.8%, jaundice--in 12.5% patients. Decompensated hepatic cirrhosis (Child-Pugh C) at first hospitalisation was diagnosed in 11.7% cases. The earliest and most significant biochemical markers of PBS were high activity of gammaglutamil-transpeptidase (GGTP) and alkaline phosphatase (AP). IgM level was high in 70.5% cases. Antimitochondrial antibodies were detected in 83.6% examinees. Overlapping with autoimmune hepatitis was seen in 9.4%. One-third of the patients had systemic symptoms. Quality of life deteriorated. CONCLUSION: PBC may be long asymptomatic. The absence of skin pruritus is not a sure sign of the disease absence. To detect PBC early, young and middle-aged women with unexplained weakness or skin pruritus should undergo a comprehensive examination including measurements of GGTP, AP, AMA, IgM, morphological investigation of liver tissue. PBC patients had low quality of life. This should be accounted for when treating patients, in determination of their working ability and for early referral for liver transplantation.     

The laparoscopic staging of primary biliary cirrhosis.

The laparoscopic photographs of 38 cases of primary biliary cirrhosis with high titres of mitochondrial antibodies were reviewed. The quality of laparoscopic photography is now so high that it proved possible to use these photographs as the basis of a system of laparoscopic staging. By analogy with the system of histological classification, the disease was divided into four stages. Laparoscopy provides an alternative approach to the diagnosis of primary biliary cirrhosis, which should be based on clinical, biochemical, immunological and morphological data. However, by laparoscopy alone it is improssible to separate pure primary biliary cirrhosis from its mixed forms with chronic active hepatitis.