As a secondary endocrine organ, the liver is closely related to the endocrine system. Liver involvement is not uncommon in endocrine diseases, such as hyper/hypothyroidism, diabetes, dysfunction of adrenal and gonadal. It can be manifested in a variety of forms, including hepatocyte injury (elevated transaminase), bile duct injury (cholestasis), hepatocyte steatosis, vascular injury and liver tumor. Direct and indirect liver injury caused by abnormal hormone levels and side effects of drugs for the treatment of endocrine diseases are common pathogenesis. In addition, endocrine diseases can be concomitant with liver diseases, such as autoimmune thyroiditis and autoimmune hepatitis. Systemic diseases can also involve the endocrine system and liver at the same time, such as systemic lupus erythematosus and IgG4 related diseases. For patients with unexplained liver injury, endocrine system diseases should be considered as the differential diagnosis.
Cholestasis/pathology* ; Endocrine System Diseases/pathology* ; Hepatitis, Autoimmune/pathology* ; Humans ; Liver/pathology* ; Liver Diseases/pathology*

Autoimmune Diseases ; diagnosis ; immunology ; pathology ; therapy ; Child ; China ; Gastroenterology ; Hepatitis B ; diagnosis ; pathology ; therapy ; Humans ; Infant

Chemical and Drug Induced Liver Injury ; pathology ; Hepatitis, Autoimmune ; pathology ; Humans ; Warfarin ; adverse effects

Autoimmune hepatitis (AIH) is a severe globally distributed liver disease that could occur at any age. Human menstrual blood-derived stem cells (MenSCs) have shown therapeutic effect in acute lung injury and liver failure. However, their role in the curative effect of AIH remains unclear. Here, a classic AIH mouse model was constructed through intravenous injection with concanavalin A (Con A). MenSCs were intravenously injected while Con A injection in the treatment groups. The results showed that the mortality by Con A injection was significantly decreased by MenSCs treatment and liver function tests and histological analysis were also ameliorated. The results of phosphoproteomic analysis and RNA-seq revealed that MenSCs improved AIH, mainly by apoptosis and c-Jun N-terminal kinase/mitogen-activated protein signaling pathways. Apoptosis analysis demonstrated that the protein expression of cleaved caspase 3 was increased by Con A injection and reduced by MenSCs transplantation, consistent with the TUNEL staining results. An AML12 co-culture system and JNK inhibitor (SP600125) were used to verify the JNK/MAPK and apoptosis signaling pathways. These findings suggested that MenSCs could be a promising strategy for AIH.
Mice ; Animals ; Humans ; Hepatitis, Autoimmune/pathology* ; Signal Transduction ; Disease Models, Animal ; Stem Cells

BACKGROUNDTo explore the clinical and pathological characteristics and pathogenesis of autoimmunohepatitis (AIH).

METHODSThe serum and liver biopsy specimens and clinical data of 26 cases with patients with AIH were analyzed and scored according to the criteria of International autoimmune hepatitis (IAIHG, 1999). The changes of dendritic cells (DC) in the liver tissues were observed with a panel of DC markers (CD-80/B7-1, CD-86/B7-2, CD-1a and HLA-DR) and immunohistochemistry, and the activation of hepatic stellate cells (HSC) and the expression of TGF-alpha were also detected. Liver tissue specimens from 10 patients with chronic viral hepatitis B and C respectively and 5 normal liver specimens were chosen as controls.

RESULTSMean aggregate scores of 26 AIH cases, including 21 cases of type B (80.8%) and 5 cases of type C (19.2%), which were 18.6 +/- 1.4 and 19.1 +/- 2.1 respectively. There were significant differences between the type B and type C in the average age levels of serum ALT and AST, and alpha-Glo (P <0.001 or P< 0.01 or P <0.05). Histological features of all the AIH liver tissues showed the lesions of chronic active hepatitis such as interface hepatitis/piecemeal necrosis (100%), obvious lobular inflammation (type B 95.2%, type C 100%), bridging necrosis (57.1% type B, 80.0% type C, P<0.05), rosetting of liver cells (71.4% type B, 100% type C, P<0.01), central lobular confluent necrosis (33.3% type B, 80.0% type C, P<0.001), predominant plasmacytic infiltration (type B 95.2%, type C 20.0%, P<0.001). The rates of increased and concentrated DC in the portal and lobular areas, especially in the active lesions in type B and type C AIH were 85.7% (18/21) and 5/5 respectively. It was found that DC and lymphocytes surrounded the hepatocytes which partly expressed HLA-DR antigen, while there were no or a few HLA-DR positive hepatocytes in controls. Meanwhile, the number of alpha-SMA positive HSC and the expression of TGF- were obviously increased in AIH liver tissues.

CONCLUSIONSSeveral clinical and pathological features of AIH were identified in this study. As an antigen-presenting cell, DC might play an important role in the pathogenesis of AIH. In China, sub-type B of AIH might be more frequent than sub-type C and there were differences in clinical aspects, serology and pathology between the two types.

Adolescent ; Adult ; Child ; Dendritic Cells ; immunology ; Female ; Hepatitis, Autoimmune ; blood ; pathology ; Humans ; Liver ; pathology ; Male ; Middle Aged

UNLABELLEDTo explore the clinical and pathological features of male and female autoimmune hepatitis (AIH) patients.

METHODSOne hundred and sixty-nine AIH patients were enrolled. The clinical and histological data of the male cases were compared with the female ones.

RESULTSThere were 23 (13.6%) male patients in our study. The general status, biochemical and immunological test, and histological findings between two groups had no significant difference (P more than 0.05). The IAIHG's revised original scoring system pretreatment scores of male patients (14.4+/-2.3) were lower than that of female ones (16.6+/-2.6, Z= -3.728, P=0.000), whereas the simplified scoring system scores of male patients (7.2+/-0.8) were higher than that of female ones (6.5+/-1.2, Z=-2.372, P=0.018). There were 15 male AIH patients treated with immunosuppressive therapy, then 12 of them reached complete biochemical remission, the other three cases were incomplete response. The complete biochemical remission rate in our male cases was 80%. Median duration of remission was 3 months (95% CI 2.070-3.930 months).

CONCLUSIONThere are no significant differences in clinical and pathological features of AIH between genders. The diagnosis of AIH should be suspected in male patients with any abnormality in serum aminotransferases levels. Liver biopsy examination is recommended to establish the diagnosis of AIH. The simplified criteria have good diagnostic value for male AIH patients.

Adolescent ; Adult ; Aged ; Female ; Hepatitis, Autoimmune ; pathology ; Humans ; Liver ; pathology ; Male ; Middle Aged ; Sex Factors ; Young Adult

Autoimmune Diseases ; drug therapy ; pathology ; Cholangitis ; drug therapy ; immunology ; pathology ; Cholangitis, Sclerosing ; drug therapy ; immunology ; pathology ; Hepatitis, Autoimmune ; drug therapy ; immunology ; pathology ; Humans ; Interferon-gamma ; therapeutic use ; Liver Cirrhosis, Biliary ; drug therapy ; immunology ; pathology ; Ursodeoxycholic Acid ; therapeutic use

Chronic HCV infection has been reported to be associated with several extrahepatic conditions such as cryoglobulinemia, lymphoma, lichen planus, porphyria cutanea tarda, autoimmune thyroiditis, and renal disease. Glomerular disease may occur in patients with chronic HCV infection. The most common patterns are membranoproliferative glomerulonephritis, and less frequently, membranous nephropathy, fibrillary glomerulonephritis and immunotactoid glomerulopathy. Few crescentic glomerulonephritis has been reported in association with HCV infection, and no case was reported in Korea yet. We experienced one case of rapidly progressive glomeruloinephritis with the pathology of crescentic glomerulonephritis complicated in membranoproliferative glomerulonephritis in a patient with HCV infection.
Cryoglobulinemia ; Glomerulonephritis* ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Hepacivirus* ; Hepatitis C* ; Hepatitis* ; Humans ; Korea ; Lichen Planus ; Lymphoma ; Pathology ; Porphyria Cutanea Tarda ; Thyroiditis, Autoimmune

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

Acute-Phase Proteins ; metabolism ; Animals ; Female ; Hepatitis, Autoimmune ; immunology ; metabolism ; pathology ; Interleukin-17 ; metabolism ; Lipocalin-2 ; Lipocalins ; metabolism ; Liver ; pathology ; Mice ; Mice, Inbred BALB C ; Oncogene Proteins ; metabolism