Junko Takiguchi

A case of hepatic inflammatory pseudotumor with primary biliary cirrhosis.

Inflammatory pseudotumor (IPT) of the liver is an unusual non-neoplastic benign lesion. A 75-year-old man was hospitalized for esophageal varices and gastric cancer. Three years before admission, he had been diagnosed as having primary biliary cirrhosis (PBC) without Sjögrens syndrome. Computed tomography (CT) and magnetic resonance imaging (MRI) scans showed multiple masses (S3, S5, S6) less than 2 cm in diameter in the liver. Since these masses were difficult to distinguish from hepatocellular carcinoma, or metastatic liver carcinoma, one of the masses (S5) was removed during an operation for gastric cancer. Histological examination demonstrated marked infiltration of plasma cells and some histiocytes, findings consistent with the histological features of IPT. The coexistence of hepatic IPT and PBC in this case may have been an accidental event. However, the immunological and environmental factors associated with PBC are thought to be involved in the development of IPT; in addition, cholangitis associated with PBC could have contributed to the development of IPT.

Two Cases of Primary Biliary Cirrhosis Complicated With Syringomyelia

1. Johnson SJ, Mathew J, MacSween RN, et al. Post-infantile giant cell hepatitis: Histological and immunohistochemical study. J Clin Pathol 1994;47:1022–7. 2. Phillips MJ, Blendis LM, Poucell S, et al. Syncytial giant-cell hepatitis. Sporadic hepatitis with distinctive pathological features, a severe clinical course, and paramyxoviral features. N Engl J Med 1991;324:455–60. 3. Pappo O, Yunis E, Jordan JA, et al. Recurrent and de novo giant cell hepatitis after orthotopic liver transplantation. Am J Surg Pathol 1994;18:804–13. 4. Roberts E, Ford-Jones EL, Phillips MJ. Ribavirin for syncytial giant cell hepatitis. Lancet 1993;341:640–1. 5. Durand F, Degott C, Sauvanet A, et al. Subfulminant syncytial giant cell hepatitis: Recurrence after liver transplantation treated with ribavirin. J Hepatol 1997;26:722–6. 6. Hassoun Z, N’Guyen B, Cote J, et al. A case of giant cell hepatitis recurring after liver transplantation and treated with ribavirin. Can J Gastroenterol 2000;14:729–31.

A case of autoimmune hepatitis in which Raynaud's phenomenon developed after occurrence of anticentromere antibody

症例は72歳の女性. 1990年1月, 肝機能障害と黄疸のため当科入院となった. 肝炎ウイルスマーカーは陰性. 抗核抗体が320倍と陽性で, 高γグロブリン血症と肝生検の結果から自己免疫性肝炎 (AIH) の診断でプレドニゾロン, アザチオプリンを投与され肝機能は改善した. 1990年1月入院時の抗核抗体の染色パターンは均一・斑紋 (homogeneous/speckled) 型を示していたが, 1997年1月には抗セントロメア抗体を示す散在性斑紋型に変化し, さらに同年2月からこれまで認められなかったレイノー現象が出現した. この間の肝機能は正常範囲で安定していた. AIHの経過中に抗核抗体の染色パターンが変化した報告はなく, さらにセントロメア抗体の出現後にその抗体と関連が指摘されているRaynaud現象が出現しており, 抗核抗体とその症状の出現の経過を追えた貴重な症例と考られた.

Role of CPG DNA in concanavalin A-induced hepatitis in mice

OBJECTIVE To investigate the effects of an intradermal injection of oligodeoxynucleotides (ODNs) containing unmethylated CpG motifs on concanavalin A (Con A)-induced hepatitis, an experimental model of immune-mediated hepatitis. METHODS Con A was injected intravenously into Balb/c mice. Twelve hours after Con A challenge, blood and liver samples were obtained. CpG ODN was injected intradermally 48 hours before Con A challenge. The extent of liver injury was assessed by determining serum alanine transaminase (ALT) and by liver histology. Serum levels of cytokines, including interferon (IFN)-gamma, tumor necrosis factor (TNF)-alpha, interleukin (IL)-4 and IL-5, were measured by enzyme-linked immunosorbent assay. RESULTS Co-administration of Con A and CpG ODN significantly increased serum ALT in mice compared with that in the case of administration of Con A alone (10,268 +/- 4,654 and 1,140 +/- 832 IU/1, respectively, p<0.05). In liver histology, mice treated with CpG ODN and Con A showed more extensive midzonal necrosis than did mice treated with Con A alone. These mice also showed significant increases in serum TNF-alpha and IFN-gamma and decrease in serum IL-5. CONCLUSIONS The results indicate that CpG ODNs aggravate Con A-induced hepatitis by stimulating the production of T-helper-1 (Th1) cytokines, TNF-alpha and IFN-gamma, suggesting that bacterial DNA that contains unmethylated CpG motifs may contribute to the exacerbation of immune-mediated liver injury.