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01.06.2015

Extrahepatic Autoimmune Conditions Associated with Primary Biliary Cirrhosis

verfasst von: Annarosa Floreani, Irene Franceschet, Nora Cazzagon, Alice Spinazzè, Alessandra Buja, Patrizia Furlan, Vincenzo Baldo, M. Eric Gershwin

Erschienen in: Clinical Reviews in Allergy & Immunology | Ausgabe 2-3/2015

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Abstract

There is a paucity of information on extrahepatic autoimmune (EHA) conditions associated with primary biliary cirrhosis (PBC) and on the impact of EHA conditions on PBC patients’ survival. Our goal was to assess the association between PBC and other autoimmune diseases and the impact of EHA conditions on the natural history of PBC. We took advantage of 361 consecutive PBC patients enrolled between 1975 and 2012 (22 males, 339 females; mean follow-up 8 ± 6.9 years). Any associated EHA conditions, PBC histological stage at diagnosis, biochemical data, physiological history, and extrahepatic malignancies developing during the follow-up were recorded. Survival was analyzed by means of Kaplan-Meier curves. Importantly, 221 patients (61.2 %) had at least one EHA conditions: 45 patients (20.4 %) had Hashimoto thyroiditis; 7 (3.2 %) had Graves’ thyroiditis; 65 (29.4 %) had Raynaud’s phenomenon; 124 (56.1 %) had Sjogren’s syndrome; 8 (3.6 %) had systemic lupus erythematosus; 22 (9.9 %) had scleroderma; 22 (9.9 %) had rheumatoid arthritis; 18 (8.1 %) had cutaneous autoimmune diseases; 8 (3.6 %) had vasculitis; 5 (1.4 %) had celiac disease; and 25 (13.1 %) had other EHA conditions. The proportion of patients with associated EHA conditions enrolled during representative periods (1975–1980, 1981–1990, 1991–2000, 2001–2010, 2011–2012) remained stable. No differences emerged between patients with versus without EHA conditions in terms of mean age at PBC diagnosis, antimitochondrial antibody (AMA), or antinuclear antibody (ANA) positivity, histological stage at diagnosis, smoking habits, alcohol consumption, or BMI >25. Multiple logistic regression analysis showed that only female gender was significantly associated with positivity for EHA conditions (OR 4.8; 95 % CI 1.6–13.7, p = 0.004). The mean survival after the diagnosis of PBC was much the same in patients with and without EHA conditions. In conclusion, EHA conditions are often associated with PBC, especially in female patients, but they do not reduce patient survival.
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Metadaten
Titel
Extrahepatic Autoimmune Conditions Associated with Primary Biliary Cirrhosis
verfasst von
Annarosa Floreani
Irene Franceschet
Nora Cazzagon
Alice Spinazzè
Alessandra Buja
Patrizia Furlan
Vincenzo Baldo
M. Eric Gershwin
Publikationsdatum
01.06.2015
Verlag
Springer US
Erschienen in
Clinical Reviews in Allergy & Immunology / Ausgabe 2-3/2015
Print ISSN: 1080-0549
Elektronische ISSN: 1559-0267
DOI
https://doi.org/10.1007/s12016-014-8427-x

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