Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease. In the 21st century, the management of ursodeoxycholic acid (UDCA)-refractory cases is the main highlight in the medical treatment of PBC. Although prescriptions, such as bezafibrate or obeticholic, are used to address refractoriness to UDCA, the development of next-generation drugs and the evaluation of the efficacy of a dual add-on therapy remain in progress, which aimed to eliminate occurrence of refractory response to add-on therapy. Other highlights include the diagnosis of anti-mitochondria antibody-negative PBC and PBC-autoimmune hepatitis overlap, prediction of treatment-response and prognosis, and management of patient-reported outcomes (pruritus).
Herein, an overview of the progress and unmet needs in the diagnosis and treatment of PBC, focusing on personalized medicine, is hereby elucidated.
Before the project related to the Career Support and Diversity Promotion in the 44th meeting of the eastern branch of Japan Society of Hepatology, a questionnaire survey on career support, role of academic societies, and social networking was conducted among conference participants. The respondents included 185 men and 53 women, including 49 (21%) aged <40 years. Many respondents believed that the lack of time (61%) and inadequate support systems (43%) were obstacles to their careers. Many respondents were positive about their degrees and studying abroad. Web-based holding of scientific meetings (74%) and web-based qualification renewal procedures (51%) were the most common requests to academic societies for career support and promotion of diversity. In addition, 44% of the respondents were on Facebook and 32% were on Twitter.
The hospital started hepatitis medical coordinator activities in 2019. From 2019 to 2021, the main effort was to recommend hepatitis C virus-positive patients to see a specialist by writing a message using the alert function of the electronic medical record to the ordering physician, however the effectiveness of this effort was limited. In 2022, the "Hepatitis Team" consisting of members from various professions, was established, and a "Flowchart for hepatitis virus marker positive patients" was created to standardize the subsequent measures. Through the immediate checkup of hepatitis virus marker-positive patients and monthly pickups by the "Hepatitis Team" the number of cases in which test results were not communicated to hepatitis C virus-positive patients decreased significantly. We believe that the hepatitis medical coordinator activities using the "Flowchart for hepatitis virus marker positive patients" prepared by the hospital functioned effectively through multidisciplinary cooperation.
We present a case of biliary bleeding caused by gallbladder perforation caused by a tumor that occurred immediately after combination therapy with atezolizumab and bevacizumab for advanced hepatocellular carcinoma. The male patient in his 70s with advanced hepatocellular carcinoma was treated with combination therapy of atezolizumab and bevacizumab on day 1 and experienced epigastralgia on day 2. On day 3, we determined that biliary obstruction had occurred due to biliary bleeding caused by gallbladder perforation caused by the tumor, and we performed biliary drainage via emergency endoscopic retrograde cholangiopancreatography. To treat tumor bleeding from the tumor, we used hepatic artery embolization of tumor blood vessel A6 on day 6. Bevacizumab was thought to have caused the biliary hemorrhage, so the combination therapy of atezolizumab and bevacizumab was stopped, lenvatinib was introduced, and subsequent CT revealed tumor shrinkage.
We report the case of a female patient in her 50s who had a 16 ×14-mm hepatic tumor, as revealed during a medical checkup. She had no remarkable medical history, and a preoperative blood test revealed no abnormalities. While abdominal ultrasonography revealed a 16 × 14-mm tumor in S3 of the liver, no enhancement was observed on contrast-enhanced computed tomography. Moreover, T1-, T2-, and diffusion-weighted magnetic resonance imaging showed only low intensity, iso-high intensity, and slightly high intensity hepatic tumor, respectively. Furthermore, no 18-fluorodeoxyglucose uptake was observed in the liver. As a diagnostic treatment, laparoscopic partial hepatic resection was performed. Pathologically, the lesion showed extensive coagulative necrosis, wherein epithelioid histiocytes were arranged in a palisading way in its periphery, and the lesion was thus diagnosed as palisading granuloma. Special staining performed to determine the cause of palisading granuloma did not reveal any pathogen. As preoperative diagnosis of palisading granuloma is difficult, laparoscopic hepatectomy may be considered a useful diagnostic treatment.
Hepatitis medical care coordinators (HMCCs) in Japan are trained by local governments and regional core centers, and are expected to play an active role in various aspects of hepatitis countermeasures. A 2019 survey revealed varied activity statuses of HMCCs among facilities. This study surveyed the present status of HMCCs in 21 of the 72 regional core centers in the fiscal year 2021.
A total of 951 HMCCs were trained at these 21 facilities. The 17 participating centers of the 2019 survey indicated a slight increase in the proportion of HMCCs who actively contributed to hepatitis patient care, from 84.2% to 85.8%.
Despite the COVID-19 pandemic, HMCCs remained active in many facilities.
We report a case of hepatocellular carcinoma with high mesenchymal-epithelial transition (MET) gene amplification demonstrating significant tumor shrinkage after cabozantinib treatment. A male patient in his fifties had far-advanced hepatocellular carcinoma and had previously been treated with combination therapy of lenvatinib or atezolizumab plus bevacizumab but had experienced progressive disease. We conducted FoundationOne® CDx, which is a comprehensive genomic profiling test, considering the next-line efficacious treatment options, and we confirmed high copy MET gene amplification. Hence, we administered cabozantinib, which caused a significant reduction in the tumor size. This case highlights the potential of comprehensive genomic profiling in identifying efficacious drugs and emphasizes the importance of actively conducting genomic testing to improve treatment outcomes.
A questionnaire survey of medical institutions Chiba Prefecture, Japan, specializing in liver diseases was conducted. This study aimed to provide an overview of the current hepatitis virus tests situations and measures taken to link test-positive individuals to specialists. The positivity rate in these institutions was high, with 2.2% for hepatitis C antibody (HCVAb) and 0.9% for hepatitis B surface antigen (HBsAg). Although many institutions (70%) had been employing linking measures, the consultation rate with hepatologists was low, with 7.6% and 14.3% for HCVAb- and for HBsAg-positive cases, respectively. Only half of the institutions disclosed that their measures were working well. These data suggested the importance of improving the system for determining test-positive individuals and promoting hepatologist consultation.