A 63-year-old woman with no significant medical history was referred to our hospital for further investigation of elevated liver enzyme levels and thrombocytopenia. The patient had a few days' history of general malaise and purpura of her legs. She had no fever or any abdominal complaints. She had a history of alcohol intake of about 40 g/day and no constant drug use.
Her vital signs were stable and physical findings were normal except for jaundice and purpura of her legs. The laboratory findings were as follows: total bilirubin, 8.8 mg/dL; aspartate aminotransferase (AST), 1,767 U/L; alanine aminotransferase (ALT), 1,845 U/L; gamma-glutamyl transpeptidase, 2,229 U/L; alkaline phosphatase (ALP), 845 U/L; immunoglobulin G (IgG), 2,042 mg/dl; anti-nuclear antibody (ANA) titer, positive at 80-fold dilution; platelet count, 22,000/muL; platelet-associated IgG (PAIgG), 208 ng/10 7 cells. Serologic markers for hepatitis A, B, C, and E viruses, and Epstein-Barr virus, cytomegalovirus, varicella zoster virus, and herpes simplex virus were all negative, and anti-Helicobacter pylori (H. pylori) IgG was positive (Table 1). Abdominal ultrasonography and enhanced computed tomography revealed no significant biliary tract disease that could have led to liver damage.
According to the criteria of the International Autoimmune Hepatitis Scoring System, the patient's pretreatment clinical score without histology was 13 (female: +2; ALP/ALT ratio: +2; IgG level: +1; ANA titer: +2, antimitochondrial antibody: 0; viral markers: +3; drugs: +1; alcohol: 0; immune disease: +2), indicating probable AIH. Her severe thrombocytopenia was considered to be due to concomitant ITP because of her clinical and laboratory findings.
Liver biopsy via the transjugular route (TJLB) was selected to confirm the diagnosis of AIH to avoid the risk of hemorrhage after percutaneous liver biopsy (PLB) in a patient with severe thrombocytopenia. The right internal jugular vein was punctured after administering local anesthesia, and a 5-Fr catheter cannulated into the right hepatic vein (RHV) over the guide wire. A venogram was performed to confirm the position of the catheter in the RHV (Figure 1(a)). The catheter was then exchanged for a transjugular liver access and biopsy set catheter (Cook Medical, Bloomington, IN). Five liver biopsy specimens were obtained. All steps were performed using X-ray fluoroscopy to confirm the location of the guide wire or catheter. A postbiopsy venogram revealed no complications, including intraperitoneal hemorrhage (Figure 1(b)). Manual compression of the access site on her neck was applied from catheter removal until hemostasis was achieved.
The histological findings of liver biopsy showed interface hepatitis and moderate to severe inflammatory infiltrates including lymphocytes and plasma cells. Moderate fibrosis in the portal area and partial destruction of the hepatic lobules with partial piecemeal necrosis was also observed (Figures 2(a), 2(b), 2(c), and 2(d)). All these pathological findings were compatible with the diagnosis of AIH, and the postbiopsy score was 17, thereby confirming the diagnosis of AIH. Bone marrow aspiration revealed a normal nucleated cell count and a slight increase in megakaryocytes, suggestive of ITP (Figure 3). She received an intravenous glycyrrhizin-containing herbal medicine, Stronger Neo-Minophagen C (SNMC; Minophagen Pharmaceutical, Tokyo, Japan) at 60 ml/day before TJLB, and prednisolone (PSL) at 55 mg/day (0.8 mg/kg/day) after the histological diagnosis of AIH. After PSL administration, the patient demonstrated a good response with restored liver enzyme levels and platelet counts (Figure 4). PSL was tapered to 5 mg/day as a maintenance dose. The patient recovered uneventfully except for a hyperglycemic event requiring insulin treatment.