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

Adult ; Aged ; Female ; Hepatitis, Autoimmune ; blood ; complications ; drug therapy ; pathology ; Humans ; Liver Cirrhosis, Biliary ; blood ; complications ; drug therapy ; pathology ; Male ; Middle Aged ; Retrospective Studies

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

BACKGROUNDLupus hepatitis is yet to be characterized based on its clinical features and is often difficult to differentially diagnose from other liver diseases. We aimed to elucidate clinical, histopathological and immunopathological features of lupus hepatitis and to evaluate primarily the effectiveness of liver immunopathological manifestations on differential diagnosis of lupus hepatitis from other liver diseases.

METHODSA retrospective study was performed to analyze clinical features of lupus hepatitis in 47 patients out of 504 inpatients with systemic lupus erythematosus (SLE) in First Affiliated Hospital of Sun Yat-sen University, China from May 2006 to July 2009, and to evaluate the association between lupus hepatitis and SLE activity. Additionally, liver histopathological changes by hematoxylin and eosin (HE) staining and immunopathological changes by direct immunofluorescence test in 10 lupus hepatitis cases were analyzed and compared to those in 16 patients with other liver diseases in a prospective study.

RESULTSOf 504 SLE patients, 47 patients (9.3%) were diagnosed to have lupus hepatitis. The prevalence of lupus hepatitis in patients with active SLE was higher than that in those with inactive SLE (11.8% vs. 3.2%, P < 0.05). The incidence of hematological abnormalities in patients with lupus hepatitis was higher than that in those without lupus hepatitis (40.4% vs. 21.7%, P < 0.05), such as leucocytes count (2.92×10(9)/L vs. 5.48×10(9)/L), platelets count (151×10(9)/L vs. 190×10(9)/L), serum C3 and C4 (0.34 g/L vs. 0.53 g/L; 0.06 g/L vs. 0.09 g/L) (P < 0.05); 45 of 47 (95.7%) lupus hepatitis patients showed 1 upper limit of normal (ULN) < serum ALT level < 5 ULN. The liver histopathological features in patients with lupus hepatitis were miscellaneous and non-specific, similar to those in other liver diseases, but liver immunopathological features showed positive intense deposits of complement 1q in 7/10 patients with lupus hepatitis and negative complement 1q deposits in all patients with other liver diseases (Fisher's exact test, P = 0.011).

CONCLUSIONSLupus hepatitis was not infrequent in active SLE patients which would be one of the indices indicating SLE activity. Positive intense deposit of complement 1q in liver may be a characteristic immunopathological feature of lupus hepatitis, which provides a new way to differentially diagnose lupus hepatitis from other liver diseases.

Adolescent ; Adult ; Aged ; Child ; Cohort Studies ; Complement C1q ; analysis ; Female ; Hepatitis, Autoimmune ; etiology ; immunology ; pathology ; Humans ; Liver ; pathology ; Lupus Erythematosus, Systemic ; complications ; Male ; Middle Aged ; Retrospective Studies

Drugs, Chinese Herbal ; therapeutic use ; Female ; Hepatitis, Autoimmune ; complications ; drug therapy ; pathology ; Humans ; Liver ; pathology ; physiopathology ; Liver Cirrhosis, Biliary ; complications ; drug therapy ; pathology ; Liver Function Tests ; Middle Aged ; Syndrome

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

Advances in the treatment of malignant and inflammatory diseases have developed over time, with increasing use of chemotherapeutic and immunosuppressive agents of a range of drug classes with varying mechanism and potency in their effects on the immune system. These advances have been met with the challenge of increased risk of hepatitis B virus (HBV) reactivation in susceptible individuals. The magnitude of risk of HBV reactivation is associated with the individual's HBV serological status and the potency and duration of immunosuppression. Individuals with chronic hepatitis B (CHB) and previously infected but serologically cleared HBV infection are both susceptible to HBV reactivation. HBV reactivation in the setting of immunosuppression is a potentially life threatening condition leading to liver failure and death in extreme cases. It is important to recognize that HBV reactivation in the setting of immunosuppression is potentially preventable. Therefore, identification of patients at risk of HBV reactivation and institution of prophylactic antiviral therapy prior to initiation of immunosuppression is essential.
Antiviral Agents/therapeutic use ; Autoimmune Diseases/complications/pathology ; Hematopoietic Stem Cell Transplantation ; Hepatitis B/complications/drug therapy ; Hepatitis B Core Antigens/blood ; Hepatitis B Surface Antigens/blood ; Hepatitis B virus/*physiology ; Humans ; Immunosuppressive Agents/therapeutic use ; Organ Transplantation ; Virus Activation/*physiology

Advances in the treatment of malignant and inflammatory diseases have developed over time, with increasing use of chemotherapeutic and immunosuppressive agents of a range of drug classes with varying mechanism and potency in their effects on the immune system. These advances have been met with the challenge of increased risk of hepatitis B virus (HBV) reactivation in susceptible individuals. The magnitude of risk of HBV reactivation is associated with the individual's HBV serological status and the potency and duration of immunosuppression. Individuals with chronic hepatitis B (CHB) and previously infected but serologically cleared HBV infection are both susceptible to HBV reactivation. HBV reactivation in the setting of immunosuppression is a potentially life threatening condition leading to liver failure and death in extreme cases. It is important to recognize that HBV reactivation in the setting of immunosuppression is potentially preventable. Therefore, identification of patients at risk of HBV reactivation and institution of prophylactic antiviral therapy prior to initiation of immunosuppression is essential.
Antiviral Agents/therapeutic use ; Autoimmune Diseases/complications/pathology ; Hematopoietic Stem Cell Transplantation ; Hepatitis B/complications/drug therapy ; Hepatitis B Core Antigens/blood ; Hepatitis B Surface Antigens/blood ; Hepatitis B virus/*physiology ; Humans ; Immunosuppressive Agents/therapeutic use ; Organ Transplantation ; Virus Activation/*physiology

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