Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis

Abstract

Summary Autoimmune cholestatic liver diseases are rare hepato-biliary disorders characterized by a progressive, inflammatory destruction of bile ducts. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the main autoimmune cholestatic liver diseases. Both may evolve into secondary biliary cirrhosis and its complications. Therapeutic options are limited and liver transplantation remains the only definitive treatment for PBC and PSC. Most PBC and PSC patients have a typical presentation, which does not require liver biopsy. However, in routine clinical practice, important variants or specific subgroups that benefit from liver biopsy for proper management may be observed. Herein, we provide a general overview of clinical and pathological characteristic of PBC and PSC, highlighting the most important features for routine diagnostic practice.

Quantitative Detection of 15 Serum Bile Acid Metabolic Products by LC/MS/MS in the Diagnosis of Primary Biliary Cholangitis

“…Histologically, PBC was characterized by bright bile duct lesions, damage of bile duct epithelial cells (BEC) and loss of intrahepatic small bile ducts, accompanied by obvious doorway infiltration, including CD4 and CD8T cells, B cells, macrophages, eosinophils and natural killer cells [12] . Usually combined with clinical features such as fatigue and itching, and abnormal biochemical and immune indexes to assist the diagnosis of PBC, [13] however, the research on early bile acid metabolic products of PBC was almost blank. The biomarker group of 8 bile acids (DCA, GDCA, LCA, GUDCA, TLCA, TUDCA, TDCA, GCDCA) was found in bile acid metabolic products.…”

Cholestasis: exploring the triangular relationship of gut microbiota-bile acid-cholestasis and the potential probiotic strategies

“…The serological hallmark of PBC is the presence of anti-mitochondrial antibodies (AMA), which is confirmed in approximately 95% of PBC patients. 26 …”

Dyslipidemia and its features in primary biliary cholangitis

“…Первичный билиарный холангит (ПБХ), ранее известный как первичный билиарный цирроз, представляет собой хроническое прогрессирующее холестатическое гранулематозное и деструктивное воспалительное поражение внутридольковых и септальных мелких желчных протоков, которое, вызвано аутоиммунным механизмом и сопровождается наличием в сыворотке крови антимитохондриальных антител (АМА) и тенденцией к прогрессированию в цирроз печени [12,31,36]. Дефицит аутоиммунной толерантности является инициирующим критическим фактором начала заболевания и постепенного развития внутрипеченочного холестаза [34]. Внутрипеченочный холестаз при ПБХ -это многофакторный процесс, который связан с повреждением субклеточных структур в эпителиоцитах внутрипеченочных желчных протоков и изменением метаболизма желчных кислот за счет нарушения процессов желчевыделения и их энтерогепатической циркуляции.…”