Autoimmune liver disorders and small-vessel vasculitis: four case reports and review of the literature

作者：Tovoli Francesco^*; Vannini Antonio; Fusconi Marco; Frisoni Magda; Zauli Danieta

来源：Annals of Hepatology, 2014, 13(1): 136-141.

DOI：10.1016/s1665-2681(19)30915-9

• Primary biliary cirrhosis
• Autoimmune hepatitis
• Anti-neutrophil cytoplasmic antibodies
• Systemic vasculitis

Autoimmune liver diseases (AILD) are a group of immunologically induced hepatic disorders that can lead to liver cirrhosis and end-stage liver disease. Extra-hepatic involvement and association with rheumatic diseases (such as Sjogren's syndrome, systemic sclerosis and rheumatoid arthritis) are well known, whereas the coexistence of AILD with small-vessel vasculitis in the same patients have been only occasionally reported. In the present paper we report four such cases and an extensive review of the literature. Clinical features of autoimmune-liver diseases associated with small-vessel vasculitis are discussed, as well as possible common pathogenic pathways including HLA genomics, costimulatory molecules and autoantibodies. In conclusion, knowledge about this association can help physicians in recognising and treating an aggressive disease which could otherwise result in severe and multiple organ damage, compromising the overall prognosis and the indication to liver transplantation.

• 出版日期2014-2