Hepatic Necrosis Mimicking Infiltrative Masses in Acute Budd-Chiari Syndrome With Hereditary Protein C Deficiency.

We report the case of a patient with an unusual acute Budd-Chiari syndrome (BCS). The patient presented with high-grade fever and right upper quadrant pain. Infiltrative lesions at the right hepatic lobe and segment IVB with intrahepatic inferior vena cava and right hepatic vein thrombus appeared on abdominal imaging. Liver biopsy revealed hepatic infarction compatible with acute BCS. Thrombophilia work-up demonstrated low protein C activity with the -1657C/T mutation of the PROC gene. Necrotic liver mass with acute BCS related to congenital protein C deficiency was diagnosed. Patient symptoms and necrotic masses improved after anticoagulant treatment for 4 months.

Endoscopic ultrasound guided fine needle biopsy (EUS-FNB) from peritoneal lesions: a prospective cohort pilot study.

BACKGROUND: Diagnostic laparoscopy is often a necessary, albeit invasive, procedure to help resolve undiagnosed peritoneal diseases. Previous retrospective studies reported that EUS-FNA is feasible on peritoneal and omental lesions, however, EUS-FNA provided a limited amount of tissue for immunohistochemistry stain (IHC). AIM: This pilot study aims to prospectively determine the effectiveness of EUS-FNB regarding adequacy of tissue for IHC staining, diagnostic rate and the avoidance rate of diagnostic laparoscopy or percutaneous biopsy in patients with these lesions. METHODS: From March 2017 to June 2018, patients with peritoneal or omental lesions identified by CT or MRI at the King Chulalongkorn Memorial Hospital, Bangkok, Thailand were prospectively enrolled in the study. All Patients underwent EUS-FNB. For those with negative pathological results of EUS-FNB, percutaneous biopsy or diagnostic laparoscopy was planned. Analysis uses percentages only due to small sample sizes. RESULTS: A total of 30 EUS-FNB passes were completed, with a median of 3 passes (range 2-3 passes) per case. For EUS-FNB, the sensitivity, specificity, PPV, NPV and accuracy of EUS-FNB from peritoneal lesions were 63.6%, 100%, 100%, 20% and 66.7% respectively. Adequate tissue for IHC stain was found in 25/30 passes (80%). The tissues from EUS results were found malignant in 7/12 patients (58.3%). IHC could be done in 10/12 patients (83.3%). Among the five patients with negative EUS results, two underwent either liver biopsy of mass or abdominal paracentesis, showing gallbladder cancer and adenocarcinoma. Two patients refused laparoscopy due to advanced pancreatic cancer and worsening ovarian cancer. The fifth patient had post-surgical inflammation only with spontaneous resolution. The avoidance rate of laparoscopic diagnosis was 58.3%. No major adverse event was observed. CONCLUSIONS: EUS-FNB from peritoneal lesions provided sufficient core tissue for diagnosis and IHC. Diagnostic laparoscopy can often be avoided in patients with peritoneal lesions.

Autoimmune hepatitis in human immunodeficiency virus-infected patients: A case series and review of the literature.

BACKGROUND: Abnormal liver chemistry is a common problem in human immunodeficiency virus (HIV)-infected patients. Common causes of abnormal liver enzymes in this population include viral hepatitis B/C or opportunistic infection, drug toxicity, and neoplasm. Autoimmune hepatitis is a rare cause of hepatitis in HIV-infected individuals; however, this condition has been increasingly reported over the past few years. CASE SUMMARY: We present 13 HIV-infected patients (5 males and 8 females) who developed autoimmune hepatitis (AIH) after their immune status was restored, i.e. all patients had stable viral suppression with undetectable HIV viral loads, and median CD4+ counts of 557 cells/× 106 L. Eleven patients presented with chronic persistent elevation of aminotransferase enzyme levels. One patient presented with acute hepatitis and the other patient presented with jaundice. The median levels of aspartate aminotransferase and alanine aminotransferase enzymes were 178 and 177 U/mL, respectively. Elevation of immunoglobulin G levels was present in 11 (85%) patients. Antinuclear antibody and anti-smooth muscle antibody were positive in 11 (85%) and 5 (38%) patients. Liver biopsy was performed in all patients. They had histopathological findings compatible with AIH. The patients were started on prednisolone for remission induction, with good response. After improvement of the liver chemistry, the dose of prednisolone was tapered, and azathioprine was added as life-long maintenance therapy. At the last follow-up visit, all were doing well, without HIV viral rebound or infectious complications. CONCLUSION: This report underscores the emergence of autoimmune hepatitis in the context of HIV infection.

IgG4-related pseudo-tumor of the kidney and multiple organ involvement mimicked malignancy.

Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized systemic condition characterized by particular clinical, serologic, and pathologic features that are consistent across a wide range of organ systems. Herein, we present a rare case of IgG4-RD presenting as multiple inflammatory pseudotumors involving the kidney and other organs involvement mimicking urothelial cell carcinoma with liver, lymph node and lung metastasis. The final diagnosis was made based on characteristic histopathological finding and analysis of IgG4 immunostaining that can distinguish from other conditions. Greater awareness of this disease is needed to ensure diagnoses, which can prevent unnecessary surgical intervention.