In this retrospective case study, we investigated the efficacy of ursodeoxycholic acid (UDCA) and prednisolone (PSL) in 20 patients with primary biliary cholangitis (PBC), having concomitant features of autoimmune hepatitis, a hepatic form of PBC. In all patients, UDCA with/without bezafibrate was administered; serum levels of AST and ALP significantly decreased in 12 patients (60%). High levels of AST, ALT, ALP and IgG were identified as significant factors associated with a poor response to UDCA. Seven patients received PSL, and all of them achieved a biochemical response. No relapse was observed, along with tapering of PSL. In conclusion, more than half of patients with a hepatic form of PBC responded to UDCA therapy, and PSL was effective for non-responders to UDCA. Some serum markers were identified for factors suggesting requirement of PSL therapy. These findings warrant further studies to verify their impact to clinical practice.

Liver cirrhosis (LC) presents with a variety of immunological abnormalities and is associated with a decrease in lymphocytes. Recently, immunodeficiency and immunodeficiency-induced bacterial infection and inflammation in LC have been labeled cirrhosis-associated immune dysfunction (CAID), and have attracted much attention, as they are associated with worsening of liver disease. In this study, we investigated total lymphocyte counts (TLC) in 274 cases of LC. In addition, we developed a stage classification for CAID diagnosis based on the neutrophil fraction (%) of leukocytes and TLC. The results of multivariate analysis revealed that leukocyte count, splenomegaly, hepatocellular carcinoma, and neutrophilia were factors contributing to the decrease in TLC, and TLC were independent prognostic factors of LC. CAID staging significantly stratified the survival rate of LC. Immunodeficiency of LC contributed to CAID-induced worsening of liver disease as a trigger of inflammation, indicating the usefulness of staging by hemogram.

The patient was a 67-year-old-woman, referred to our institution with prolonged slight fever and weight loss. Blood tests revealed an increased inflammatory response and hepatic disorder. Abdominal ultrasound demonstrated a mass lesion with cystic lesions in the liver. Contrast-enhanced CT showed a 10-cm in diameter mass in the right lobe of the liver; the solid part of the mass exhibited a contrast enhancement effect. Based on the clinical course and imaging findings, a hepatic abscess was initially considered, but malignancy could not be ruled out. We therefore performed a liver biopsy, which revealed infiltration of inflammatory cells including foamy histocytes. Eventually, the pathological diagnosis proved to be a xanthogranulomatous mass. Xanthogranulomatous tumors are sometimes difficult to distinguish from malignant tumors because of the difficulty in obtaining characteristic image findings, and surgical resection is often selected. In this case, percutaneous liver biopsy was useful to diagnose and avoid unnecessary liver resection.

A 59-year-old man with advanced non-small-cell lung cancer (NSCLC) developed acute liver injury 35 days after receiving pembrolizumab. There was no evidence of another cause for the hepatitis. Liver biopsy revealed interface hepatitis and CD8-positive lymphocyte infiltration in the portal area and liver parenchyma. Pembrolizumab-induced liver injury, which is a type of immune-related adverse event (irAE), was diagnosed. He received a combination of corticosteroid and mycophenolate mofetil (MMF), as his condition was refractory to corticosteroids. He then he received atezolizumab treatment for NSCLC. Eight days after initiation of atezolizumab, he relapsed. As the histological findings were similar to the first liver biopsy, he was diagnosed with atezolizumab-induced liver injury. A combination of corticosteroid and MMF achieved remission. The advisability of rechallenging with immune checkpoint inhibitors in patients with a history of severe irAE is controversial. We recommend careful monitoring of such patients to ensure early detection of irAE.

A 68-year-old man was admitted to our hospital with disturbance of consciousness. He had no history of liver disease. Laboratory evaluation revealed an elevated serum ammonia of 176 μg/dl. Hyperammonemia was not improved by oral administration of Lactitol hydrate. Computed tomography and ultrasound examination demonstrated a shunt between the left portal vein and the left hepatic vein, for which percutaneous transhepatic obliteration to the portal-hepatic venous shunt was performed. Following the shunt embolization, hyperammonemia was improved.

Hepatic encephalopathy due to a portal-hepatic venous shunt is relatively rare. When a patient presents with hyperammonemia and no history of liver disease, it is necessary to explore for the presence of a portosystemic venous shunt.

A 72-year-old woman with lung metastases and right adrenal metastasis from hepatocellular carcinoma was treated with lenvatinib. Progressive disease was observed on computed tomography (CT) 12 weeks after the initial treatment with lenvatinib. Two days later, she complained of a sudden onset of right upper quadrant pain; the CT showed retroperitoneal hemorrhage due to right adrenal metastasis. Emergent angiography displayed the extravasation and transcatheter arterial embolization (TAE) was performed from the right inferior phrenic artery. The patient's abdominal pain improved gradually, and she was discharged 18 days after TAE. We retrospectively reviewed the CT findings 12 weeks following the initial treatment with lenvatinib, showing that an area of high attenuation was apparent on the caudal side of the tumor, suggesting intratumoral hemorrhage. The close screening for presence of intratumoral hemorrhage was required during lenvatinib treatment.

A 59 year-old-Japanese female was referred to our hospital for tumor-induced abdominal pain. Dynamic computed tomography (CT) demonstrated a tumor, 88×73 mm in diameter, which was adherent to the caudate lobe of the liver and pancreas. Based on these findings, we performed a laparotomy with a preoperative diagnosis of hepatocellular carcinoma (HCC) originating from the caudate lobe of the liver. The tumor could be dissected from the liver, however, it had infiltrated into the common hepatic artery, leading to incomplete extirpation of the tumor. Histological examination revealed that the tumor was an ectopic HCC. Lenvatinib mesylate (lenvatinib) was used as systemic therapy for growth of the remnant tumor 16 days after surgery. CT performed during two months after initiating lenvatinib revealed that the tumor size had reduced with a decrease in vascularity, which was deemed to be a partial response. CT performed three months after the initiation of lenvatinib demonstrated regrowth of the tumor. The patient died of HCC 164 days after surgery.

A 24-year-old Vietnamese woman, with no significant medical history, presented to our hospital with right hypochondrial pain and fever. Laboratory examination showed elevated levels of hepatobiliary enzymes and marked eosinophilia. CT scan findings demonstrated polycystic low-density areas of both liver lobes. The laboratory data and diagnostic imaging suggested some variety of parasitic infection, however no parasite eggs were detected. We diagnosed fascioliasis due to a prominent positive reaction for fasciola antigen by immunoserological tests. Following administration of praziquantel (total 9 g) the laboratory data normalized, but CT scan findings showed that hepatic and extrahepatic lesions were temporarily enlarged. Both lesions shrunk six months later. This clinical course was of interest as the sequential CT scan findings did not match the patient's symptoms and laboratory results.