Auto-immune hepatitis is a chronic necroinflammatory liver disorder that is characterized by hypergammaglobulinemia, auto-antibodies in serum, and, on histological examination, the presence of periportal hepatitis. Although it can be associated with a number of other auto-immune diseases, Sjogren's syndrome is rarely associated with auto-immune hepatitis. We herein report an unusual case of auto-immune hepatitis associated with primary Sjogren's syndrome. A 39-year-old woman was admitted to our hospital due to jaundice. Laboratory data showed negative viral hepatitis marker, increased serum IgG level, positive anti- nuclear antibody, and an increased rheumatoid factor titer. The patient had no history of taking medications and alcohol. Based on characteristic clinical features, liver biopsy findings, positive Schirmer's test, and salivary scintigraph, she was diagnosed as having auto-immune hepatitis and Sjogren's syndrome. The patient achieved complete remission with steroid monotherapy.
Adult ; Hepatitis, Autoimmune/*complications/diagnosis ; Humans ; Sjogren's Syndrome/*complications/diagnosis

Autoantibodies ; Cholangitis, Sclerosing ; complications ; diagnosis ; Hepatitis, Autoimmune ; complications ; diagnosis ; Humans ; Liver Cirrhosis, Biliary ; complications ; diagnosis ; Syndrome

Autoantibodies ; blood ; Cholangitis, Sclerosing ; complications ; diagnosis ; Hepatitis, Autoimmune ; complications ; diagnosis ; Humans ; Liver Cirrhosis, Biliary ; complications ; diagnosis

Autoimmune hepatitis (AIH) is a chronic necroinflammatory liver disease of unknown cause associated with circulating autoantibodies and high serum globulin level. Systemic lupus erythematosus (SLE) is a disease of unknown etiology in which tissues and cells are damaged by pathogenic autoantibodies and immune complex, affecting multiple organs including the liver, kidney, and CNS. The difference between the hepatic involvement of SLE and autoimmune hepatitis has not been clearly defined in the past due to similarities in clinical and biochemical features. A scoring system for the diagnosis of AIH has been established, and AIH and SLE-associated hepatitis have been defined as two different entities, although both have the same autoimmune features such as polyarthralgia, hypergammaglobulinemia and circulating autoantibodies. AIH has been considered to occur infrequently in SLE. We report two cases of AIH which simultaneously satisfied the criteria of SLE.
Adult ; Female ; Hepatitis, Autoimmune/*complications/diagnosis ; Humans ; Lupus Erythematosus, Systemic/*complications/diagnosis ; Middle Aged

Cholangitis, Sclerosing ; complications ; diagnosis ; therapy ; Hepatitis, Autoimmune ; complications ; diagnosis ; therapy ; Humans ; Liver Cirrhosis, Biliary ; complications ; diagnosis ; therapy ; Syndrome

Overlap of autoimmune hepatitis and systemic lupus erythematosus (SLE) is a comparatively rare condition. Although both autoimmune hepatitis and SLE can share common autoimmune features such as polyarthralgia, hypergammaglobulinemia and positive ANA, it has been considered as two different entities. We report a case of anti-LKM1 positive autoimmune hepatitis who developed SLE two years later. The presence of interface hepatitis with lymphoplasma cell infiltrates and rosette formation points to the autoimmune hepatitis rather than SLE hepatitis. Autoimmune hepatitis is infrequently accompanied by SLE, therefore, it could be recommended to investigate for SLE in patients with autoimmune hepatitis.
Antibodies, Antinuclear/analysis ; Autoantibodies/*analysis ; Echocardiography ; Female ; Hepatitis, Autoimmune/complications/*diagnosis/immunology ; Humans ; Liver/pathology ; Lupus Erythematosus, Systemic/complications/*diagnosis/immunology ; Young Adult

OBJECTIVETo analyze the clinical and pathological characteristics of primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) overlap syndrome.

METHODSOf our 68 patients with the diagnosis of PBC, we identified 9 overlap syndrome cases strictly using the revised descriptive criteria and scoring system for diagnosis of AIH proposed by the International Autoimmune Hepatitis Group Report. The clinical manifestations and pathological changes shown in liver biopsies from the overlap syndrome and pure PBC were analyzed and compared.

RESULTSThe mean aggregate scores of the 9 cases (13.2%) of the overlap syndrome group and 59 cases of pure PBC (86.8%) were 10.2+/-0.2 and 4.7+/-0.7 respectively among the 68 total. The serum levels of ALT and AST, immunoglobulin G, gammaglobulin, in the overlap syndrome group were significantly higher than those in the pure PBC group, and also there was a frequent presence of antinuclear antibody and/or smooth muscle antibody positivity in the overlap syndrome group. Histopathologically the livers in the overlap syndrome group showed combined features of interface hepatitis and piecemeal necrosis, characterizing the two diseases.

CONCLUSIONThe overlap syndrome group showed combined features of both PBC and AIH. There were differences in clinical aspects, serology and histology between the overlap syndrome and pure PBC groups.

Adult ; Female ; Hepatitis, Autoimmune ; complications ; diagnosis ; pathology ; Humans ; Immunoglobulin G ; blood ; Liver ; pathology ; Liver Cirrhosis, Biliary ; complications ; diagnosis ; pathology ; Male ; Middle Aged ; Retrospective Studies

Visceral leishmaniasis (VL) is a life-threatening infection caused by Leishmania species. In addition to typical clinical findings as fever, hepatosplenomegaly, and cachexia, VL is associated with autoimmune phenomena. To date, VL mimicking or exacerbating various autoimmune diseases have been described, including systemic lupus erythematosus (SLE), rheumatoid arthritis, and autoimmune hepatitis (AIH). Herein, we presented a patient with VL who had overlapping clinical features with SLE, AIH, as well as antimitochondrial antibody (AMA-M2) positive primary biliary cirrhosis.
Adult ; Autoantibodies/blood ; Bone Marrow/pathology ; Cytological Techniques ; Diagnosis, Differential ; Female ; Hepatitis, Autoimmune/complications/*pathology ; Histocytochemistry ; Humans ; Leishmaniasis, Visceral/complications/*diagnosis/*pathology ; Liver/pathology ; Liver Cirrhosis, Biliary/complications/*pathology ; Lupus Erythematosus, Systemic/complications/*pathology

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

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