title: Autoimmunity and HCV infection in porphyria cutanea tarda: a controlled study.
abstract: Autoimmunity and high rates of autoantibodies have been implicated in the pathogenesis of porphyria cutanea tarda. These abnormalities could be in part virus-induced, since porphyria cutanea tarda in most geographical regions is highly associated with hepatitis C virus infection. We analyzed the link of autoantibodies, autoimmune hepatitis and systemic lupus erythematosus in 111 patients with porphyria cutanea tarda and sex- and age-matched controls (mean age 58+/-13 years) in Germany, a region with a low prevalence of hepatitis C virus infection. Patients with porphyria cutanea tarda displayed lower rates of anti-nuclear antibodies (16/111, 14% vs 28/111, 25%, p<0,05) and of antibodies against smooth muscle (25/111, 23% vs 48/111, 43%, p<0,01), than controls. The percentage of patients with porphyria cutanea tarda with positive anti-HCV was low but significantly higher than in our controls (9/111, 8% vs 0/111, 0%, respectively), (p<0,05). Two patients with porphyria cutanea tarda (2/111, 2%) fulfilled the criteria for systemic lupus erythematosus and not one of 65 patients was found to have clinical autoimmune hepatitis. In the first controlled study of a large cohort of patients with porphyria cutanea tarda no increased prevalence of selected autoantibodies and autoimmune hepatitis was found. However, a higher prevalence of HCV infection and systemic lupus erythematosus in patients with porphyria cutanea tarda was confirmed.
authors.affiliation: Department of Gastroenterology, Medizinische Klinik II, Klinikum Chernitz GmbH, Teaching Hospital of the University of Leipzig, Germany. u.stoelzel@skc.de
authors.forename: Ulrich
authors.initials: U
authors.lastname: Stölzel
authors.forename: Detlef
authors.initials: D
authors.lastname: Schuppan
authors.forename: Hans L
authors.initials: HL
authors.lastname: Tillmann
authors.forename: Michael P
authors.initials: MP
authors.lastname: Manns
authors.forename: Andrea
authors.initials: A
authors.lastname: Tannapfel
authors.forename: Manfred O
authors.initials: MO
authors.lastname: Doss
authors.forename: Thomas
authors.initials: T
authors.lastname: Zimmer
authors.forename: Erich
authors.initials: E
authors.lastname: Köstler
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000974:Antibodies, Antinuclear / Q000097:blood; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000150:complications; D016022:Case-Control Studies; D005260:Female; D005858:Germany / Q000453:epidemiology; D006526:Hepatitis C / Q000150:complications*; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D009130:Muscle, Smooth / Q000276:immunology; D017119:Porphyria Cutanea Tarda / Q000209:etiology* / Q000276:immunology / Q000821:virology; D015995:Prevalence
chemical_list: D000974:Antibodies, Antinuclear; D001323:Autoantibodies