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Objective@#To examine the effects of ursodeoxycholic acid combined with Traditional Chinese Medicine on biochemical response in patients with primary biliary cholangitis.@*Methods@#According to the method of receiving treatment, 197 patients with primary biliary cholangitis were divided into Traditional Chinese Medicine plus Western medicine group (93 cases, 47.2%) and Western medicine group (104 cases, 52.8%). From the baseline date, the combined group was treated with ursodeoxycholic acid plus traditional Chinese medicine decoction or Chinese patent medicine for at least one month and the Western medicine group simply took ursodeoxycholic acid . Additionally, Traditional Chinese medicine decoction prescriptions were mainly Xiaoyaosan and Yinchenhao. Chinese patent medicine were restricted to Biejia Ruangan tablets, Fuzheng Huayu capsules, Jiuweigantai capsules and Yinzhihuang capsules, which were used to treat liver fibrosis and cholestasis. The primary efficacy endpoint was defined as ALP level < 1.67 × ULN and ≥ 15% decrease in ALP with baseline level and TBIL≤ULN after 12 months of treatment.@*Results@#The overall biochemical response rate of patients was 35.0% (69/197). The response rate of TCM+ Western medicine group was 43.0% (40/93), and that of Western medicine group was 27.9% (29/104). The difference between the two groups was statistically significant (χ2 = 4.936, P < 0.05). Further analysis showed that the Chinese and Western medicine group was superior to the Western medicine group alone in reducing γ-glutamyltransferase (GGT) and TBiL [the median decline were GGT: 160.1 U/L and 111.3 U/L (Z = -2.474, P < 0.05), TBiL: 5.2 umol/l and 3.1 umol/l (Z = -2.125, P < 0.05)].@*Conclusion@#UDCA combined with TCM therapy can remarkably improve the biochemical response rate in patients with PBC and distinctly decrease the TBIL and GGT levels than UDCA monotherapy.
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Objective To investigate the clinical features of autoimmune liver disease (AILD) complicated by abdominal lymphadenectasis (LA),as well as the clinical significance of LA in AILD.Methods A retrospective analysis was performed for the clinical data of 252 patients who were admitted to The First Affiliated Hospital of Dalian Medical University from January 2005 to April 2016,and among these patients,52 had autoimmune hepatitis (AIH),174 had primary biliary cholangitis (PBC),and 26 had AIH-PBC overlap syndrome (AIH-PBC OS).A total of 78 patients underwent follow-up.According to the presence or absence of LA,these patients were divided into LA group with 70 patients and non-LA (NLA) group with 182 patients.As for general information,laboratory markers,imaging findings,and follow-up results,the normally distributed continuous data were expressed as mean ± SD,and the t-test was used for comparison between groups;the non-normally distributed continuous data were expressed as median,and the Wilcoxon rank sum test was used for comparison between groups.The categorical data were expressed as rates,and the chi-square test,the corrected chi-square test,or the Fisher's exact test was used for comparison of these data between groups.Results There were no significant differences between the two groups in age,sex ratio,proportion of patients who were diagnosed due to abnormal liver function found by physical examination,and incidence rates of other autoimmune diseases.The incidence rate of abdominal LA was 22％ (11/52) in the AIH group,26.4％ (46/174) in the PBC group,and 50％ (13/26) in the AIH-PBC OS group,and the AIH-PBC OS group had a significantly higher incidence rate than the AIH group and the PBC group (x2 =7.693,P =0.021).The LA group had significantly higher levels of alkaline phosphatase and gamma-glutamyl transpeptidase (GGT) than the NLA group (Z =2.944 and 3.169,P =0.003 and 0.002).For the patients with PBC,the LA group had a significantly higher level of GGT than the NLA group (Z =2.136,P =0.033);for the patients with AIH-PBC OS,the LA group had a significantly higher level of total bilirubin thau the NLA group (Z =2.121,P =0.035);for the patients with AIH,there were no significant differences in these indices between the LA group and the NLA group.The LA group had a higher incidence rate of abnormal imaging findings than the NLA group (97.1％ vs 81.9％,x2 =9.863,P =0.002).A total of 78 patients were followed up with a median follow-up time of 18 months.Of all patients in the LA group,6 (23.1％) achieved complete remission,7 (26.9％) achieved incomplete response,1 (3.8％) experienced recurrence,and 12 (46.2％) experienced treatment failure;of all patients in the NLA group,21 (40.4％) achieved complete remission,17 (32.7％) achieved incomplete response,7 (13.5％) experienced recurrence,and 7(13.5％) experienced treatment failure;there was a significant difference in the distribution of treatment outcomes between the LA group and the NLA group (Z =2.406,P =0.016).Conclusion Patients with AIH-PBC OS have a higher incidence rate of abdominal LA than those with AIH and PBC,and patients with AILD complicated by LA may have marked cholestasis and liver impairment and poor treatment response,suggesting that LA might be used to determine disease severity and judge prognosis.
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Primary biliary cholangitis (PBC) is a chronic cholestatic autoimmune disease caused by the attack of the liver by the immune system and is commonly seen in middle-aged women.The pathological features of this disease include the infiltration of a large number of innate immune cells (NK cells,NK T cells,and monocytes,etc.) and adaptive immune cells (T lymphocytes and B lymphocytes) at the portal area and injury of small intrahepatic bile ducts.In addition,PBC patients have high serum levels of anti-mitochondrial antibodies and inflammatory cytokines such as interferon-γ,tumor necrosis factor-α,interleukin-6,and interleukin-12.Although current studies have shown that autoreactive T cells are the major effector cells for PBC,NK cells,NK T cells,monocytes,and B cells around the portal vein also directly or indirectly participate in the development and progression of PBC.This article systematically summarizes the role of innate immune cells,adaptive immune cells,and related chemokines in the pathogenesis of PBC,in order to provide a theoretical basis for a deep understanding of the immunological pathogenesis of PBC.
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In recent years,ursodeoxycholic acid is commonly used for the treatment of primary biliary cholangitis (PBC);however,the growing number of PBC patients and the occurrence of suboptimal response and treatment intolerance pose a great challenge to treatment regimens.The approval of the new drug obeticholic acid brings hope to PBC patients,and a combination of fibrates also has a promising future.More studies are in progress.Although new drugs,such as monoclonal antibody,fibroblast growth factor 19,and sodium-dependent bile acid transporter inhibitor,have limited efficacy data,they provide new directions for the treatment of PBC.With the help of individualized follow-up and stratified therapy,the management of PBC patients will enter a new stage.
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Primary biliary cholangitis (PBC) is an autoimmune liver disease with strong genetic susceptibility.The genome-wide association studies and immunochip studies conducted in recent years help to reveal the pathogenesis of PBC.The susceptibility genes of PBC are classified into human leukocyte antigen gene and non-human leukocyte antigen gene,and most of the susceptibility loci are associated with immune regulation,suggesting that disorders of the immune regulatory pathways may play an important role in the pathogenesis of PBC.In addition,the weighted genetic risk score of these candidate genes may predict the risk of PBC.At present,about one third of PBC patients have suboptimal response to ursodeoxycholic acid;therefore,targeted drugs for susceptibility genes may become an effective substitutive therapy.
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Primary biliary cholangitis (PBC) is a disease with relatively strong heterogeneity,and different patients may have great differences in clinical process and phenotypes.Therefore,long-term treatment and follow-up are necessary for accurate risk stratification of these patients.Observation of clinical symptoms of PBC patients,measurement of the changes in related indices,and individualized management for each patient helps to provide standard treatment for low-risk PBC patients and bring benefits to high-risk patients through well-designed clinical studies.
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Primary biliary cholangitis (PBC) is an autoimmune liver disease mainly involving the interlobular bile ducts and can progress to liver cirrhosis and liver failure.Early diagnosis and management can significantly improve the prognosis of such patients and their quality of life.This article focuses on the achievements of PBC research and related difficult issues in China,with reference to the clinical practice guidelines for PBC by European Association for the Study of the Liver in 2017 and experience in clinical practice.
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An ideal animal model of primary biliary cholangitis (PBC) plays an important role in the research on physiopathological mechanism and drug research and development.In recent years,great achievements have been made in animal models which can reflect the features of PBC,such as positive serum anti-mitochondrial antibody and immunopathological injury of the bile duct.There are various methods for the preparation of animal models of PBC at present,including chemical or biological exposure and gene knockout.However,these models cannot completely simulate PBC in humans,since they have different serological,immunological,and histopathological features.This suggests the complexity of pathological mechanisms of PBC,including gene specificity and environmental changes and helps us to understand the pathogenesis of events in the early stage of PBC,such as the break of immune tolerance and specific attack of biliary epithelial cells.
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Platynosomum fastosum is a parasite that affects cats, and among these, domestic cats, especially those originating from areas with tropical and subtropical climates. It is the most common liver parasite of domestic cats and is usually located in the bile ducts and gall bladder, but may also be found in the small intestine and pancreas. Infection occurs through ingestion of geckos containing metacercariae, causing cholangitis in the bile ducts of domestic cats. The aim of this study was to report the occurrence of P. fastosum after necropsy of an asymptomatic domestic cat, with a history of sudden death, received at the Animal Pathology sector of the Ricardo Alexandre Hippler Veterinary Hospital, Espírito Santo, Brazil. In macroscopic examination adult specimens of P. fastosum were observed inside the visibly dilated bile ducts. The dilatation, the presence of parasites and papillary proliferations into the lumen of the ducts were confirmed by histopathological examination. Then, a cholangiohepatitis associated to P. fastosum infection was classified. This is the first report of the occurrence of P. fastosum in a domestic cat in the state of Espírito Santo