Factors associated with response to therapy and outcome of patients with primary biliary cirrhosis with features of autoimmune hepatitis

Background & Aims: For patients with primary biliary cirrhosis (PBC) with features of autoimmune hepatitis (AIH), treatment with ursodeoxycholic acid (UDCA) alone or in combination with immunosuppression is controversial. Little is known about the factors associated with initial response to therapy or outcome. We performed a retrospective analysis of treatment strategies and factors associated with outcomes of patients with PBC-AIH. Methods: We analyzed data from 88 patients who were diagnosed with PBC-AIH according to Paris criteria, from 7 centers in 5 countries. First-line therapies included UDCA alone (n= 30) or a combination of UDCA and immunosuppression (n= 58). Results: Of patients who received UDCA alone as the first-line therapy, 37% did not respond to treatment. Severe interface hepatitis was independently associated with lack of response to treatment (P= .024; odds ratio, 0.05; 95% confidence interval, 0.004-0.68). The combination of UDCA and immunosuppression was effective in 73% of patients who had not been previously treated or had not responded to UDCA. The presence of advanced fibrosis was associated with lack of response to the combination of UDCA and immunosuppression (P= .003; odds ratio, 0.13; 95% confidence interval, 0.03-0.48). Second-line immunosuppressive agents (cyclosporine, tacrolimus, and mycophenolate mofetil) led to biochemical remission in 54% of patients who did not respond to initial immunosuppression. Liver transplants were given to 4patients with PBC-AIH. Five patients died during follow-up (3 from liver-related causes). Conclusions: In a retrospective study of a large cohort of patients with PBC-AIH, UDCA alone did not produce a biochemical response in most patients with severe interface hepatitis; these patients require additional therapy with immunosuppression. Second-line immunosuppressive agents are effective in controlling disease activity in patients who do not respond to conventional immunosuppression. © 2014 AGA Institute.

Yazar Ozaslan E.
Efe C.
Heurgué-Berlot A.
Kav T.
Masi C.
Purnak T.
Muratori L.
Yayın Türü Article
Tek Biçim Adres https://hdl.handle.net/20.500.12628/5864
Tek Biçim Adres 10.1016/j.cgh.2013.09.021
Konu Başlıkları Autoimmunity
Malignancy
Mycophenolate-Mofetil
Paris Criteria
Koleksiyonlar Araştırma Çıktıları | WoS | Scopus | TR-Dizin | PubMed | SOBİAD
Scopus İndeksli Yayınlar Koleksiyonu
WoS İndeksli Yayınlar Koleksiyonu
Dergi Adı Clinical Gastroenterology and Hepatology
Dergi Cilt Bilgisi 12
Dergi Sayısı 5
Sayfalar 863 - 869
Yayın Yılı 2014
Eser Adı
[dc.title]
Factors associated with response to therapy and outcome of patients with primary biliary cirrhosis with features of autoimmune hepatitis
Yazar
[dc.contributor.author]
Ozaslan E.
Yazar
[dc.contributor.author]
Efe C.
Yazar
[dc.contributor.author]
Heurgué-Berlot A.
Yazar
[dc.contributor.author]
Kav T.
Yazar
[dc.contributor.author]
Masi C.
Yazar
[dc.contributor.author]
Purnak T.
Yazar
[dc.contributor.author]
Muratori L.
Yayın Yılı
[dc.date.issued]
2014
Yayıncı
[dc.publisher]
W.B. Saunders
Yayın Türü
[dc.type]
article
Özet
[dc.description.abstract]
Background & Aims: For patients with primary biliary cirrhosis (PBC) with features of autoimmune hepatitis (AIH), treatment with ursodeoxycholic acid (UDCA) alone or in combination with immunosuppression is controversial. Little is known about the factors associated with initial response to therapy or outcome. We performed a retrospective analysis of treatment strategies and factors associated with outcomes of patients with PBC-AIH. Methods: We analyzed data from 88 patients who were diagnosed with PBC-AIH according to Paris criteria, from 7 centers in 5 countries. First-line therapies included UDCA alone (n= 30) or a combination of UDCA and immunosuppression (n= 58). Results: Of patients who received UDCA alone as the first-line therapy, 37% did not respond to treatment. Severe interface hepatitis was independently associated with lack of response to treatment (P= .024; odds ratio, 0.05; 95% confidence interval, 0.004-0.68). The combination of UDCA and immunosuppression was effective in 73% of patients who had not been previously treated or had not responded to UDCA. The presence of advanced fibrosis was associated with lack of response to the combination of UDCA and immunosuppression (P= .003; odds ratio, 0.13; 95% confidence interval, 0.03-0.48). Second-line immunosuppressive agents (cyclosporine, tacrolimus, and mycophenolate mofetil) led to biochemical remission in 54% of patients who did not respond to initial immunosuppression. Liver transplants were given to 4patients with PBC-AIH. Five patients died during follow-up (3 from liver-related causes). Conclusions: In a retrospective study of a large cohort of patients with PBC-AIH, UDCA alone did not produce a biochemical response in most patients with severe interface hepatitis; these patients require additional therapy with immunosuppression. Second-line immunosuppressive agents are effective in controlling disease activity in patients who do not respond to conventional immunosuppression. © 2014 AGA Institute.
Kayıt Giriş Tarihi
[dc.date.accessioned]
2019-12-23
Açık Erişim Tarihi
[dc.date.available]
2019-12-23
Yayın Dili
[dc.language.iso]
eng
Konu Başlıkları
[dc.subject]
Autoimmunity
Konu Başlıkları
[dc.subject]
Malignancy
Konu Başlıkları
[dc.subject]
Mycophenolate-Mofetil
Konu Başlıkları
[dc.subject]
Paris Criteria
Haklar
[dc.rights]
info:eu-repo/semantics/closedAccess
ISSN
[dc.identifier.issn]
1542-3565
İlk Sayfa Sayısı
[dc.identifier.startpage]
863
Son Sayfa Sayısı
[dc.identifier.endpage]
869
Dergi Adı
[dc.relation.journal]
Clinical Gastroenterology and Hepatology
Dergi Sayısı
[dc.identifier.issue]
5
Dergi Cilt Bilgisi
[dc.identifier.volume]
12
Tek Biçim Adres
[dc.identifier.uri]
https://dx.doi.org/10.1016/j.cgh.2013.09.021
Tek Biçim Adres
[dc.identifier.uri]
https://hdl.handle.net/20.500.12628/5864
Görüntülenme Sayısı ( Şehir )
Görüntülenme Sayısı ( Ülke )
Görüntülenme Sayısı ( Zaman Dağılımı )
Görüntülenme
5
09.12.2022 tarihinden bu yana
İndirme
1
09.12.2022 tarihinden bu yana
Son Erişim Tarihi
09 Şubat 2024 07:49
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Tıklayınız
patients immunosuppression response associated combination PBC-AIH treatment therapy respond hepatitis interval interface effective biochemical confidence agents immunosuppressive Second-line retrospective factors initial fibrosis require additional controlling activity disease advanced conventional presence responded Institute treated produce severe
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