OBJECTIVETo realize the clinical features of autoimmune hepatitis-primary biliary cirrhosis (AIH+PBC) overlap syndrome.

METHODSWe analyzed and compared the biochemistry, autoimmune antibodies, and liver biopsy results of 129 autoimmune hepatic disease cases retrospectively, using the international criteria to see which could be diagnosed as AIH/PBC overlap syndrome.

RESULTSOur 35 AIH+PBC overlap syndrome patients were mainly women, with a sex ratio of 1 female: 10 male, and a median age of 50.79+/-11.27 (20 to 70 years old). They had AIH characteristics such as flare of ALT, AST and elevated immunoglobulin G (IgG), gamma-immunoglobulin. There were also antinuclear antibodies (74.3%); moderate or severe periportal or periseptal lymphocytic infiltration, piecemeal necrosis, and florid bile duct lesions, high serum levels of ALP, presence of mitochondrial antibodies (68.6%) and M2 antibodies (45.7%), and features of PBC.

CONCLUSIONSAIH+PBC overlap syndrome is not rare. It should be diagnosed in time and to find effective treatments for it.

Adult ; Autoantibodies ; blood ; Cholagogues and Choleretics ; therapeutic use ; Female ; Hepatitis, Autoimmune ; complications ; diagnosis ; drug therapy ; Humans ; Immunoglobulin G ; blood ; Liver Cirrhosis, Biliary ; complications ; diagnosis ; drug therapy ; Male ; Middle Aged ; Retrospective Studies ; Ursodeoxycholic Acid ; therapeutic use

BACKGROUND/AIMS: Overlap syndrome of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) (AIH-PBC overlap syndrome) is a rare disease that has not been clearly characterized in Korean patients. This study investigated the clinical features of AIH-PBC overlap syndrome compared with those of AIH and PBC alone. METHODS: This retrospective cohort study included 158 consecutive patients who were diagnosed as AIH (n=61), PBC (n=81), or AIH-PBC overlap syndrome (n=9) based on the Paris and the International Autoimmune Hepatitis Group (IAIHG) criteria from 2001 to 2011 in Korea. We compared the clinical features of these three groups retrospectively, including their biochemical characteristics, treatments, responses, and clinical outcomes. RESULTS: The AIH-PBC overlap syndrome patients exhibited biochemical characteristics of both AIH and PBC, and showed a similar response to ursodeoxycholic acid (UDCA) monotherapy as for the PBC patients. However, the response of AIH-PBC overlap syndrome patients to UDCA and steroid combination therapy was worse than the response of AIH patients to steroid-based therapy (P=0.024). Liver cirrhosis developed more rapidly in AIH-PBC overlap syndrome patients than in AIH patients group (P=0.013), but there was no difference between AIH-PBC overlap syndrome patients and PBC patients. The rates of developing hepatic decompensation did not differ significantly between the groups. CONCLUSIONS: The AIH-PBC overlap syndrome patients exhibited a worse response to UDCA and steroid combination therapy and a faster cirrhotic progression compared with AIH patients.
Adult ; Aged ; Cohort Studies ; Drug Therapy, Combination ; Female ; Hepatitis, Autoimmune/complications/*diagnosis ; Humans ; Liver/metabolism/pathology ; Liver Cirrhosis, Biliary/complications/*diagnosis/drug therapy ; Male ; Middle Aged ; Republic of Korea ; Retrospective Studies ; Steroids/therapeutic use ; Treatment Outcome ; Ursodeoxycholic Acid/therapeutic use

Autoimmune hepatitis (AIH) has been reported in association with Sjogren's syndrome (SS). Drug-induced AIH has been rarely reported. A rare case of the co-development of AIH and SS in a 53-year-old woman after the consumption of herbal medicines is described. After admission, the patient complained of dryness in her mouth, and she was subsequently diagnosed with SS, which had not been detected previously. The patient's bilirubin and aminotransferase levels initially decreased following conservative management; however, they later began to progressively increase. A diagnosis of AIH was made based on the scoring system proposed by the International Autoimmune Hepatitis Group. The patient was administered a combination of prednisolone and azathioprine, and the results of follow-up liver-function tests were found to be within the normal range. This is an unusual case of AIH and SS triggered simultaneously by the administration of herbal medicines.
Alanine Transaminase/blood ; Aspartate Aminotransferases/blood ; Azathioprine/therapeutic use ; Bilirubin/blood ; Female ; Hepatitis, Autoimmune/complications/*diagnosis/drug therapy ; *Herbal Medicine ; Humans ; Liver/pathology ; Liver Function Tests ; Middle Aged ; Prednisolone/therapeutic use ; Sjogren's Syndrome/complications/*diagnosis/drug therapy

Systemic sclerosis (SSc) is a chronic systemic disease that affects the skin, lungs, heart, gastrointestinal tract, kidneys, and musculoskeletal system. Although up to 90% of patients with scleroderma have been estimated to have gastrointestinal involvement, liver disease has been reported only rarely. A 51-year-old woman was hospitalized due to esophageal variceal bleeding. Her serum was positive for anti-nuclear antibody and anti-centromere antibody. Sclerodactyly was noted on both hands, and she had recently developed Raynaud's syndrome. Punch biopsy of the hand showed hyperkeratosis, regular acanthosis, and increased basal pigmentation in the epidermis, and thick pale collagenous bundles in the dermis. Liver biopsy showed chronic active hepatitis with bridging fibrosis. Consequently, she was diagnosed with liver cirrhosis due to autoimmune hepatitis (AIH) combined with SSc. AIH had subsided after administration of prednisolone at 40 mg per day. She received 5-10 mg/day of prednisolone as an outpatient, and her condition has remained stable. Patients with either AIH or SSc should be monitored for further development of concurrent autoimmune diseases. The early diagnosis of AIH combined with SSc will be helpful in achieving optimal management.
Anti-Inflammatory Agents/therapeutic use ; Antibodies, Antinuclear/blood ; Esophageal and Gastric Varices ; Female ; Gastrointestinal Hemorrhage ; Hepatitis, Autoimmune/complications/*diagnosis/drug therapy ; Humans ; Liver Cirrhosis/*diagnosis/etiology/pathology ; Middle Aged ; Prednisolone/therapeutic use ; Raynaud Disease/diagnosis ; Scleroderma, Systemic/complications/*diagnosis ; Skin/pathology

A 37-year-old male presented with fever and jaundice was diagnosed as hepatitis A complicated with progressive cholestasis and severe autoimmune hemolytic anemia. He was treated with high-dose prednisolone (1.5 mg/kg), and eventually recovered. His initial serum contained genotype IA hepatitis A virus (HAV), which was subsequently replaced by genotype IIIA HAV. Moreover, at the time of development of hemolytic anemia, he became positive for immunoglobulin M (IgM) anti-hepatitis E virus (HEV). We detected HAV antigens in the liver biopsy specimen, while we detected neither HEV antigen in the liver nor HEV RNA in his serum. This is the first report of hepatitis A coinfected with two different genotypes manifesting with autoimmune hemolytic anemia, prolonged cholestasis, and false-positive IgM anti-HEV.
Adult ; Anemia, Hemolytic, Autoimmune/*diagnosis/drug therapy/etiology ; Anti-Inflammatory Agents/therapeutic use ; Cholestasis/*diagnosis/drug therapy/pathology ; Coinfection/*diagnosis ; Genotype ; Hepatitis A/complications/*diagnosis/genetics ; Hepatitis E/complications/*diagnosis/genetics ; Humans ; Immunoglobulin M/blood ; Liver/pathology/virology ; Male ; Prednisolone/therapeutic use ; RNA, Viral/blood

Pure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) have rarely been reported as an extrahepatic manifestation of acute hepatitis A (AHA). We report herein a case of AHA complicated by both PRCA and AIHA. A 49-year-old female with a diagnosis of AHA presented with severe anemia (hemoglobin level, 6.9 g/dL) during her clinical course. A diagnostic workup revealed AIHA and PRCA as the cause of the anemia. The patient was treated with an initial transfusion and corticosteroid therapy. Her anemia and liver function test were completely recovered by 9 months after the initial presentation. We review the clinical features and therapeutic strategies for this rare case of extrahepatic manifestation of AHA.
Acute Disease ; Adult ; Anemia, Hemolytic, Autoimmune/*complications/*diagnosis/drug therapy ; Antineoplastic Agents, Hormonal/therapeutic use ; Bone Marrow/pathology ; Female ; Hepatitis A/*complications/*diagnosis ; Humans ; Male ; Middle Aged ; Prednisolone/therapeutic use ; Red-Cell Aplasia, Pure/*complications/*diagnosis/drug therapy ; Treatment Outcome ; Young Adult

The pathogenesis of autoimmune hepatitis (AIH) is unclear, but viral infections have been proposed as a potential trigger in patients with genetic predisposition. We report a case of AIH following acute hepatitis A (AHA). A 57-year-old woman presented with fatigue and pitting edema for last 3 months. She had been diagnosed as an AHA 15 months ago based on clinical features, biochemical tests and positive HAV IgM antibody at a local clinic. Her biochemical tests was normalized one month after AHA diagnosis, but the serum levels of aminotransferase started to rise four months after AHA diagnosis. Antinuclear antibody was positive at a titer of 1:40, and anti-smooth muscle antibody was also positive. Hypergammaglobulinemia and liver pathology were typical for AIH. The patients had a score of 17 according to the International Autoimmune Hepatitis Group's system. She was given prednisolone and azathioprine and showed complete response to immunosuppressive therapy. The present case is the first report on AIH triggered by AHA in Korea.
Acute Disease ; Alanine Transaminase/blood ; Antibodies, Antinuclear/analysis ; Aspartate Aminotransferases/blood ; Autoantibodies/analysis ; Azathioprine/therapeutic use ; Female ; Hepatitis A/complications/*drug therapy ; Hepatitis, Autoimmune/*diagnosis/drug therapy/etiology ; Humans ; Hypergammaglobulinemia/diagnosis ; Immunosuppressive Agents/therapeutic use ; Liver/pathology ; Middle Aged ; Prednisolone/therapeutic use

A 25-year-old woman presented with jaundice, palpitation, and weight loss of 5 kg during a period of 2 weeks. Laboratory tests showed elevated levels of liver enzymes (AST 1,282 IU/L, ALT 1,119 IU/L) and total bilirubin (6.4 mg/dL); negative for hepatitis virus infection; elevated serum levels of triiodothyronine (T3, 3.60 ng/dL), free thyroxine (fT4, 3.82 ng/dL), and lowered serum level of thyroid stimulating hormone (TSH, <0.025 microIU/mL); and positive for thyroid stimulating antibody and anti-mitochondrial antibody (AMA). The liver biopsy findings were consistent with autoimmune hepatitis (AIH). Accordingly, oral steroid therapy was started with 60 mg of prednisolone under the impression of AIH associated with Graves' disease. After a week of steroid therapy, the clinical manifestation showed significant improvement, with normalization of both liver and thyroid functions. Diagnosis of the liver condition of patients who present with hyperthyroidism and liver dysfunction is important, so that appropriate therapy can be promptly initiated.
Adult ; Alanine Transaminase/analysis ; Antibodies, Antinuclear/blood ; Aspartate Aminotransferases/analysis ; Bilirubin/blood ; Female ; Graves Disease/complications/*diagnosis/drug therapy ; Hepatitis, Autoimmune/complications/*diagnosis/drug therapy ; Humans ; Immunoglobulins, Thyroid-Stimulating/blood ; Liver/enzymology/metabolism/pathology ; Prednisolone/therapeutic use ; Steroids/therapeutic use ; Thyrotropin/blood