[Clinicopathological features of early- and late-stage primary biliary cirrhosis: a comparative study]

【早期和晚期原发性胆汁性肝硬化的临床病理特征:一项比较研究】

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Abstract

OBJECTIVE: To investigate the clinicopathological features of different stages of primary biliary cirrhosis (PBC), and to provide a basis of evidence-based medicine for early identification and effective treatment of this disease. METHODS: A total of 130 patients with pathologically confirmed PBC and complete clinical data were enrolled and divided into early-stage group and late-stage group based on pathological results. A retrospective analysis was performed for patients' general information, clinical manifestations, laboratory examinations, and pathological changes. RESULTS: The PBC patients had a mean age of 43.5±7.1 years, with middle-aged female patients accounting for 89%. The most common symptom was fatigue, followed by jaundice, pruritus, and abdominal distension in the late stage. Of all patients, 11.5% were complicated by autoimmune disease. The level of aminotransferases tended to decrease with the progression of PBC and showed no significant differences between the two groups (P > 0.05). Most patients showed an increase in serum bilirubin, mainly direct bilirubin; serum total bilirubin and direct bilirubin tended to increase with disease progression and showed significant differences between the two groups (P < 0.01). The patients showed increases in the serum levels of alkaline phosphatase (ALP) andγ-glutamyl transpeptidase (GGT), but with the disease progression, the serum level of ALP increased and that of GGT decreased; the serum levels of ALP and GGT showed no significant differences between the early- and late-stage groups (P > 0.05). The positive rate of antimitochondrial antibody was 85%. The histopathological changes of PBC included severe lesions in the portal area and surrounding areas and slight lobular lesions. In the early stage, there were injuries of the interlobar bile ducts, proliferation of small bile ducts, aggregation and invasion of mononuclear cells in surrounding tissues, and the formation of lymphoid follicle-like structure; in the late stage, there were fibrotic expansion of the portal area, formation of fibrous septa and pseudolobuli, and even liver cirrhosis. CONCLUSION: PBC is commonly seen in middle-aged women and has an insidious onset. Early- and late-stage PBC have their own clinicopathological features. As for patients with no characteristic changes in serological test, liver biopsy should be performed to give a confirmed diagnosis and avoid missed diagnosis and misdiagnosis.

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