Changes in disease characteristics of primary biliary cholangitis: An observational retrospective study from 1982 to 2016.

age asymptomatic gender outcome primary biliary cholangitis ursodeoxycholic acid

Journal

Hepatology research : the official journal of the Japan Society of Hepatology
ISSN: 1386-6346
Titre abrégé: Hepatol Res
Pays: Netherlands
ID NLM: 9711801

Informations de publication

Date de publication:
Feb 2021
Historique:
received: 25 05 2020
revised: 14 10 2020
accepted: 23 10 2020
pubmed: 31 10 2020
medline: 31 10 2020
entrez: 30 10 2020
Statut: ppublish

Résumé

Disease characteristics of primary biliary cholangitis have changed recently. However, detailed studies on the subject have been limited. Therefore, we aimed to clarify disease characteristics of patients with recent primary biliary cholangitis using the cohort from Niigata University and 21 affiliated hospitals. Overall, 508 patients were enrolled in this study from 1982 to 2016, divided into three cohorts according to their year of diagnosis: ≤1999, 2000-2009 and ≥2010. We compared differences in clinical characteristics, response to ursodeoxycholic acid and prognosis. The male-to-female ratio increased incrementally from 1:16.4 (≤1999) to 1:3.8 (≥2010) (P < 0.001). In women, the median age at diagnosis increased incrementally from 54.0 years (≤1999) to 60.5 years (≥2010) (P < 0.001) and serum albumin decreased gradually (P = 0.001), which might have affected the increase in the Fibrosis-4 Index and albumin-bilirubin score. The ursodeoxycholic acid response rate according to the Barcelona criteria increased incrementally from 26.7% (≤1999) to 78.4% (≥2010) (P < 0.010), and those according to other criteria (Paris-I, Rotterdam and Toronto) were approximately ≥80% in all cohorts. Ten-year survival rate in the ≤1999 and 2000-2009 cohorts were 98.6% and 95.6%, respectively. These earlier cohorts were also characterized by a higher rate of asymptomatic state and mild histology (83.5% [≤1999] and 84.7% [2000-2009], and 93.6% [≤1999] and 91.1% [2000-2009]). Patients with primary biliary cholangitis were characterized by older age at diagnosis and an increase in male to female ratio as well as higher response rates of ursodeoxycholic acid and longer survival, resulting from the early recognition of primary biliary cholangitis.

Identifiants

pubmed: 33126288
doi: 10.1111/hepr.13586
doi:

Types de publication

Journal Article

Langues

eng

Pagination

166-175

Informations de copyright

© 2020 The Japan Society of Hepatology.

Références

Kaplan MM. Primary biliary cirrhosis. N Engl J Med 1996; 335: 1570-1580.
Working Subgroup (English version) for Clinical Practice Guidelines for Primary Biliary Cirrhosis. Guidelines for the management of primary biliary cirrhosis: The Intractable Hepatobiliary Disease Study Group supported by the Ministry of Health, Labour and Welfare of Japan. Hepatol Res 2014; 44(Suppl S1): 71-90.
Tanaka A, Mori M, Matsumoto K, Ohira H, Tazuma S, Takikawa H. Increase trend in the prevalence and male-to-female ratio of primary biliary cholangitis, autoimmune hepatitis, and primary sclerosing cholangitis in Japan. Hepatol Res 2019; 49: 881-889.
Poupon RE, Balkau B, Eschwège E, Poupon R. A multicenter, controlled trial of ursodiol for the treatment of primary biliary cirrhosis. UDCA-PBC Study Group. N Engl J Med 1991; 324: 1548-1554.
Iwasaki S, Ohira H, Nishiguchi S et al. The efficacy of ursodeoxycholic acid and bezafibrate combination therapy for primary biliary cirrhosis: A prospective, multicenter study. Hepatol Res 2008; 38: 557-564.
Corpechot C, Chazouillères O, Rousseau A et al. A Placebo-Controlled Trial of Bezafibrate in Primary Biliary Cholangitis. N Engl J Med 2018; 378: 2171-2181.
Honda A, Tanaka A, Kaneko T et al. Bezafibrate Improves GLOBE and UK-PBC Scores and Long-Term Outcomes in Patients with Primary Biliary Cholangitis. Hepatology 2019; 70: 2035-2046.
Chazouillères O, Wendum D, Serfaty L, Montembault S, Rosmorduc O, Poupon R. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy. Hepatology 1998; 28: 296-301.
Scheuer PJ. Primary biliary cirrhosis. Proc R Soc Med 1967; 60: 1257-1260.
Parés A, Caballería L, Rodés J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic Acid. Gastroenterology 2006; 130: 715-720.
Corpechot C, Abenavoli L, Rabahi N et al. Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis. Hepatology 2008; 48: 871-877.
Corpechot C, Chazouillères O, Poupon R. Early primary biliary cirrhosis: biochemical response to treatment and prediction of long-term outcome. J Hepatol 2011; 55: 1361-1367.
Kuiper EM, Hansen BE, de Vries RA et al. Improved prognosis of patients with primary biliary cirrhosis that have a biochemical response to ursodeoxycholic acid. Gastroenterology 2009; 136: 1281-1287.
Kumagi T, Guindi M, Fischer SE et al. Baseline ductopenia and treatment response predict long-term histological progression in primary biliary cirrhosis. Am J Gastroenterol 2010; 105: 2186-2194.
Lammers WJ, Hirschfield GM, Corpechot C et al. Development and validation of a scoring system to predict outcomes of patients with primary biliary cirrhosis receiving ursodeoxycholic acid therapy. Gastroenterology 2015; 149: 1804-1812.
Carbone M, Sharp SJ, Flack S et al. The UK-PBC risk scores: Derivation and validation of a scoring system for long-term prediction of end-stage liver disease in primary biliary cholangitis. Hepatology 2016; 63: 930-950.
Ishibashi H, Komori A, Shimoda S, Ambrosini YM, Gershwin ME, Nakamura M. Risk factors and prediction of long-term outcome in primary biliary cirrhosis. Intern Med 2011; 50: 1-10.
Trivedi PJ, Bruns T, Cheung A et al. Optimizing risk stratification in primary biliary cirrhosis: AST/platelet ratio index predicts outcome independent of ursodeoxycholic acid response. J Hepatol 2014; 60: 1249-1258.
Hayashi M, Abe K, Fujita M, Okai K, Takahashi A, Ohira H. Changes in serum levels of leucine-rich a2-glycoprotein predict prognosis in primary biliary cholangitis. Hepatol Res 2019; 49: 385-393.
Fujinaga Y, Namisaki T, Moriya K et al. Identification of clinical risk factors for histological progression of primary biliary cholangitis. Hepatol Res 2019; 49: 1015-1025.
Johnson PJ, Berhane S, Kagebayashi C et al. Assessment of liver function in patients with hepatocellular carcinoma: a new evidence-based approach-the ALBI grade. J Clin Oncol 2015; 33: 550-558.
Murillo Perez CF, Goet JC, Lammers WJ et al. Milder disease stage in patients with primary biliary cholangitis over a 44-year period: A changing natural history. Hepatology 2018; 67: 1920-1930.
Lleo A, Colapietro F. Changes in the Epidemiology of Primary Biliary Cholangitis. Clin Liver Dis 2018; 22: 429-441.
Lock RJ, Unsworth DJ. Immunoglobulins and immunoglobulin subclasses in the elderly. Ann Clin Biochem 2003; 40: 143-148.
Hirohara J, Nakano T, Seki T, Okazaki K, Nakanuma Y, Tsubouchi H. National surveys of primary biliary cirrhosis (PBC) in Japan. Nihon Shokakibyo Gakkai Zasshi 2013; 110: 8-15.
Prince MI, Chetwynd A, Craig WL, Metcalf JV, James OF. Asymptomatic primary biliary cirrhosis: clinical features, prognosis, and symptom progression in a large population based cohort. Gut 2004; 53: 865-870 Erratum in: Gut 2004; 53: 1216.
Tanaka A, Hirohara J, Nakanuma Y, Tsubouchi H, Takikawa H. Biochemical responses to bezafibrate improve long-term outcome in asymptomatic patients with primary biliary cirrhosis refractory to UDCA. J Gastroenterol 2015; 50: 675-682.
Ichida F. Primary biliary liver cirrhosis. Nihon Shokakibyo Gakkai Zasshi 1975; 72: 1428-1430.
Shi J, Wu C, Lin Y, Chen YX, Zhu L, Xie WF. Long-term effects of mid-dose ursodeoxycholic acid in primary biliary cirrhosis: a meta-analysis of randomized controlled trials. Am J Gastroenterol 2006; 101: 1529-1538.
Toda G, Tanaka N, Ikeda Y. et al.Dose-dependency of effect of ursodeoxycholic acid on primary biliary cirrhosis. A randomized, double-blind controlled study. KANTANSUI 1998; 37: 443-460.
Cheung AC, Lammers WJ, Murillo Perez CF et al. Effects of age and sex of response to ursodeoxycholic acid and transplant-free survival in patients with primary biliary cholangitis. Clin Gastroenterol Hepatol 2019; 17: 2076-2084.
Corpechot C, Carrat F, Bahr A, Chrétien Y, Poupon RE, Poupon R. The effect of ursodeoxycholic acid therapy on the natural course of primary biliary cirrhosis. Gastroenterology 2005; 128: 297-303.
Tanaka A, Hirohara J, Nakano T et al. Effect of deferred or no treatment with ursodeoxycholic acid in patients with early primary biliary cholangitis. Hepatol Res 2018; 48: 727-734.
Yagi M, Tanaka A, Abe M et al. Symptoms and health-related quality of life in Japanese patients with primary biliary cholangitis. Sci Rep 2018; 8: 12542.

Auteurs

Masaaki Takamura (M)

Division of Gastroenterology and Hepatology, Niigata University Graduate School of Medical and Dental Science, Niigata, Niigata, Japan.

Yasunobu Matsuda (Y)

Department of Medical Technology, Niigata University Graduate School of Health Sciences, Niigata, Niigata, Japan.

Naruhiro Kimura (N)

Division of Gastroenterology and Hepatology, Niigata University Graduate School of Medical and Dental Science, Niigata, Niigata, Japan.

Masafumi Takatsuna (M)

Division of Gastroenterology and Hepatology, Niigata University Graduate School of Medical and Dental Science, Niigata, Niigata, Japan.

Toru Setsu (T)

Division of Gastroenterology and Hepatology, Niigata University Graduate School of Medical and Dental Science, Niigata, Niigata, Japan.

Atsunori Tsuchiya (A)

Division of Gastroenterology and Hepatology, Niigata University Graduate School of Medical and Dental Science, Niigata, Niigata, Japan.

Akihiko Osaki (A)

Niigata City General Hospital, Niigata, Niigata, Japan.

Nobuo Waguri (N)

Niigata City General Hospital, Niigata, Niigata, Japan.

Masahiko Yanagi (M)

JA Niigata Kouseiren Ojiya General Hospital, Ojiya, Niigata, Japan.

Toru Takahashi (T)

JA Niigata Kouseiren Ojiya General Hospital, Ojiya, Niigata, Japan.

Soichi Sugitani (S)

JA Niigata Kouseiren Murakami general Hospital, Murakami, Niigata, Japan.

Yuka Kobayashi (Y)

JA Niigata Kouseiren Nagaoka Chuo General Hospital, Nagaoka, Niigata, Japan.

Akira Yoshikawa (A)

JA Niigata Kouseiren Nagaoka Chuo General Hospital, Nagaoka, Niigata, Japan.

Toru Ishikawa (T)

Saiseikai Niigata Hospital, Niigata, Niigata, Japan.

Toshiaki Yoshida (T)

Saiseikai Niigata Hospital, Niigata, Niigata, Japan.

Toshiaki Watanabe (T)

Watanabe Clinic, Sanjo, Niigata, Japan.

Hitoshi Bannai (H)

Niigata Saiseikai Sanjo Hospital, Sanjo, Niigata, Japan.

Tomoyuki Kubota (T)

Niigata Rinko Hospital, Niigata, Niigata, Japan.

Kazuhiro Funakoshi (K)

Niigata Prefectural Central Hospital, Joetsu, Niigata, Japan.

Hiroto Wakabayashi (H)

Takeda General Hospital, Aizuwakamatsu, Fukushima, Japan.

So Kurita (S)

Niigata Cancer Center Hospital, Niigata, Niigata, Japan.

Norio Ogata (N)

Niigata Cancer Center Hospital, Niigata, Niigata, Japan.

Masashi Watanabe (M)

Niigata Prefectural Shibata Hospital, Shibata, Niigata, Japan.

Yuhsaku Mita (Y)

Niigataminami Hospital, Niigata, Niigata, Japan.

Shigeki Mori (S)

Niitsu Medical Center Hospital, Niigata, Niigata, Japan.

Motoya Sugiyama (M)

Sugiyama Internal Medicine Clinic, Niigata, Niigata, Japan.

Toru Miyajima (T)

JA Niigata Kouseiren Toyosaka Hospital, Niigata, Niigata, Japan.

Sumio Takahashi (S)

JA Niigata Medical Center, Niigata, Niigata, Japan.

Shuichi Sato (S)

Kido Hospital, Niigata, Niigata, Japan.

Kisei Ishizuka (K)

Niigata Shirone General Hospital, Niigata, Niigata, Japan.

Hironobu Ohta (H)

Niigata Shirone General Hospital, Niigata, Niigata, Japan.

Yutaka Aoyagi (Y)

JA Niigata Medical Center, Niigata, Niigata, Japan.

Shuji Terai (S)

Division of Gastroenterology and Hepatology, Niigata University Graduate School of Medical and Dental Science, Niigata, Niigata, Japan.

Classifications MeSH