title: Autoimmune liver disorders and small-vessel vasculitis: four case reports and review of the literature.
abstract: 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 Sjögren'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.
authors.affiliation: University of Bologna, Department of Medical and Surgical Sciences. Bologna, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Tovoli
authors.affiliation: Azienda Ospedaliero-Universitaria S.Orsola-Malpighi Bologna, Department of Emergency. Bologna, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Vannini
authors.affiliation: Azienda Ospedaliero-Universitaria S.Orsola-Malpighi Bologna, Department of Internal Medicine and Digestive Diseases. Bologna, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Fusconi
authors.affiliation: Azienda Ospedaliero-Universitaria S.Orsola-Malpighi Bologna, Department of Internal Medicine and Digestive Diseases. Bologna, Italy.
authors.forename: Magda
authors.initials: M
authors.lastname: Frisoni
authors.affiliation: University of Bologna, Department of Medical and Surgical Sciences. Bologna, Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Zauli
mesh_terms: D000328:Adult; D000368:Aged; D015267:Churg-Strauss Syndrome / Q000150:complications / Q000276:immunology*; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000276:immunology*; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000276:immunology*; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000150:complications / Q000276:immunology*; D008297:Male; D055953:Microscopic Polyangiitis / Q000150:complications / Q000276:immunology*