4.A Case of Cholestatic Autoimmune Hepatitis and Acute Liver Failure: an Unusual Hepatic Manifestation of Mixed Connective Tissue Disease and Sjogren's Syndrome.
Jun Ki MIN ; Nam Ik HAN ; Jin A KIM ; Young Sok LEE ; Chul Soo CHO ; Ho Youn KIM
Journal of Korean Medical Science 2001;16(4):512-515
Although hepatomegaly is reported to occur occasionally in patients with mixed connective tissue disease (MCTD) or Sjogren's syndrome (SS), autoimmune liver diseases such as primary biliary cirrhosis, sclerosing cholangitis, and autoimmune hepatitis in association with MCTD or SS have rarely been described. We report a case of severe cholestatic autoimmune hepatitis presenting with acute liver failure in a 40-yr-old female patient suffering from MCTD and SS. The diagnosis of MCTD and SS was made at the age of 38. The patient presented severe jaundice and elevation of conjugated bilirubin. The patient denied alcohol and drug use and had no evidence of viral hepatitis. On the 8th day of her hospitalization, the patient developed grade III hepatic encephalopathy. She was diagnosed as autoimmune hepatitis presenting with acute liver failure based on clinical features, positive FANA and anti-smooth muscle antibodies, negative anti-mitochondrial antibodies, high titers of serum globulin, liver biopsy findings, and a good response to corticosteroid therapy, The patient was managed with prednisolone and the clinical symptoms, liver function test results, and liver biopsy findings showed much improvement after steroid therapy.
Adult
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Cholestasis/*etiology
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Female
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Hepatitis, Autoimmune/*etiology
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Human
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Liver Failure, Acute/*etiology
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Mixed Connective Tissue Disease/*complications
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Sjogren's Syndrome/*complications
5.Autoimmune hepatitis in a patient with myasthenia gravis and thymoma: a report on the first case in Korea.
Yo Seb HAN ; Byung Ho KIM ; Tae Hyung KIM ; Seok Ho DONG ; Hyo Jong KIM ; Young Woon CHANG ; Joung Il LEE ; Rin CHANG ; Youn Wha KIM ; Joo Chul PARK
The Korean Journal of Internal Medicine 2000;15(2):151-155
Myasthenia gravis is an autoimmune disease that results from an antibody-mediated reaction and occurs with thymoma in 15+ACU- of patients. It is very rarely associated with autoimmune hepatitis. Four cases of myasthenia gravis with autoimmune hepatitis have been reported in the world. We recently experienced a case of 30-year-old man with myasthenia gravis associated with thymoma and autoimmune hepatitis. This condition is the first case that has not been reported previously in Korea. We report this rare condition along with a brief review of the literature.
Adult
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Case Report
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HLA-DR Antigens/genetics
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Hepatitis, Autoimmune/etiology+ACo-
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Human
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Male
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Myasthenia Gravis/complications+ACo-
;
Thymoma/etiology+ACo-
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Thymus Neoplasms/etiology+ACo-
6.Clinical and immunopathological features of patients with lupus hepatitis.
Ru-Hua ZHENG ; Jin-Hui WANG ; Shu-Bing WANG ; Jie CHEN ; Wei-Ming GUAN ; Min-Hu CHEN
Chinese Medical Journal 2013;126(2):260-266
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
7.Fulminant hepatic failure and hepatitis B virus reactivation: case reports and analyses of the pathological mechanism.
Chinese Journal of Hepatology 2014;22(4):272-276
OBJECTIVETo investigate the clinical features and mechanisms of hepatitis B virus (HBV) reactivation induced by chemotherapy or immunosuppressive therapy and subsequent fulminant hepatic failure (FHF) in patients with autoimmune diseases.
METHODSSeven cases of FHF related to HBV reactivation were retrospectively assessed. All patients had been confirmed as hepatitis B e antigen (HBeAg) seronegative and had undergone glucocorticoid-based therapy to manage primary diseases, including nephrotic syndrome (2 cases), polycystic kidney disease combined with chronic nephritis (1 case), conditions following kidney transplantation (1 case), lymphadenoma (1 case), idiopathic thrombocytopenic purpura (1 case), and angitis (1 case). Levels of sero-markers of HBV and HBV DNA were recorded. Serum samples from patients were respectively applied to HepG2.2.15 and HepG2 cell lines in order to investigate the effects on cell proliferation (by MTT assay) and apoptosis (by Hoechst 33342 staining assay). Intergroup differences were statistically assessed by the t-test.
RESULTSFor all patients, the initial clinical signs of hepatic failure emerged at 4 to 11 months after receipt of the glucocorticoid treatment. At the time of hepatic failure, HBeAg seropositivity was detected in 4 patients, including one patient who also showed seropositivity for the hepatitis B surface antibody (HBsAb). All 7 patients showed high levels of HBV DNA when the hepatitis condition flared. Neither remedial antiviral treatments nor internal medicine comprehensive treatments, including therapeutic plasma exchange, were effective in any of these cases. The duration from clinical signs onset to death ranged from 24 to 47 days. Treatment of HepG2.2.15 and HepG2 cells with serum samples from patients with FHF showed a dosage-effect relationship of the serum concentration on the cellular proliferation inhibition rate, with the serum of patients with FHF having more severe inhibiting effects on the HepG2.2.15 cells than on the HepG2 cells. The HepG2.2.15 cells showed a greater tendency towards apoptosis upon treatment with serum samples from patients with FHF, compared to the HepG2 cells.
CONCLUSIONHBV reactivation induced by chemotherapy or immunosuppressive therapy is a problem currently encountered in the management of malignancies or rheumatic autoimmune disease patients. It is critical to verify HBV status prior to initiation of these treatment strategies so that appropriate antiviral prophylaxis may be administered, so as to reduce the risk of HBV reactivation and subsequent repression of cell proliferation and apoptosis that can promote development of FHF and increase a patient's risk of death.
Adult ; Aged ; Apoptosis ; Autoimmune Diseases ; complications ; Cell Proliferation ; Female ; Glucocorticoids ; adverse effects ; therapeutic use ; Hep G2 Cells ; Hepatitis B ; complications ; virology ; Hepatitis B virus ; drug effects ; Humans ; Liver Failure ; etiology ; virology ; Male ; Middle Aged ; Retrospective Studies ; Virus Activation ; drug effects
8.Mycophenolate mofetil as an alternative treatment for autoimmune hepatitis.
Seung Woon PARK ; Soon Ho UM ; Han Ah LEE ; Sang Hyun KIM ; Yura SIM ; Sun Young YIM ; Yeon Seok SEO ; Ho Sang RYU
Clinical and Molecular Hepatology 2016;22(2):281-285
Autoimmune hepatitis (AIH) is an immune-mediated chronic liver disease characterized by hepatocellular inflammation, necrosis, and fibrosis, which can progress to cirrhosis and fulminant hepatic failure. The standard treatment for AIH includes corticosteroids alone or in combination with azathioprine. Although most patients achieve remission using the standard regimen, some patients do not respond due to either drug intolerance or refractory disease; in such cases alternative immunosuppressive agents should be explored. The second-line therapies are cyclophilin inhibitors such as cyclosporine A or tacrolimus, and nowadays mycophenolate mofetil (MMF) is widely used if azathioprine-based therapies are not tolerated. Although these are recommended as an alternative to the first-line regimen, there is insufficient evidence for the efficacy of second-line therapies, with the evidence based mainly on expert opinion. Therefore, we report an AIH patient receiving the standard regimen in whom remission did not occur due to side effects to azathioprine, but was successfully treated with MMF in combination with corticosteroids as an alternative to the standard regimen.
Alanine Transaminase/analysis
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Alopecia/etiology
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Antibiotics, Antineoplastic/*therapeutic use
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Aspartate Aminotransferases/analysis
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Azathioprine/adverse effects
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Female
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Hepatitis, Autoimmune/*drug therapy/pathology
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Humans
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Liver/enzymology/pathology
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Middle Aged
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Mycophenolic Acid/*therapeutic use
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Pancytopenia/etiology
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Prednisolone/therapeutic use
9.Autoimmune Hepatitis: Recent Update on Diagnosis and Treatment.
The Korean Journal of Hepatology 2006;12(3):318-332
Autoimmune hepatitis (AIH) is an unresolving, predominantly periportal hepatitis that is usually displays hypergammaglobulinemia, and tissue autoantibodies, and this malady is responsive to immunosuppressive therapy. Our understanding about this clinical entity has been greatly expanded since the first description by Waldenstrom 50 years ago. The codified diagnostic criteria of AIH prepared by International Autoimmune Hepatitis Group are still valid, but new attempts are being made to overcome the shortcomings of this scoring system. Immunosuppressive therapies using prednisone and azathioprine are currently the mainstay for the treatment of AIH, but there are still many practical questions to be solved.
Adolescent
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Adult
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Aged
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Autoantibodies
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Azathioprine/administration & dosage/*therapeutic use
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Child
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Child, Preschool
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Drug Therapy, Combination
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Female
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Hepatitis, Autoimmune/*diagnosis/*drug therapy/etiology
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Humans
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Immunosuppressive Agents/administration & dosage/*therapeutic use
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Male
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Middle Aged
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Prednisone/administration & dosage/*therapeutic use
10.Liver Cirrhosis Due to Autoimmune Hepatitis Combined with Systemic Sclerosis.
Byung Chul YOU ; Soung Won JEONG ; Jae Young JANG ; So Mi GOO ; Sang Gyune KIM ; Young Seok KIM ; Chan Hong JEON ; Yoon Mi JEEN
The Korean Journal of Gastroenterology 2012;59(1):48-52
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
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Antibodies, Antinuclear/blood
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Esophageal and Gastric Varices
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Female
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Gastrointestinal Hemorrhage
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Hepatitis, Autoimmune/complications/*diagnosis/drug therapy
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Humans
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Liver Cirrhosis/*diagnosis/etiology/pathology
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Middle Aged
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Prednisolone/therapeutic use
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Raynaud Disease/diagnosis
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Scleroderma, Systemic/complications/*diagnosis
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Skin/pathology