Primary biliary cirrhosis (PBC)-autoimmune hepatitis (AIH) overlap syndrome is used to describe the coexistence of both diseases, with either a sequential or a simultaneous presentation in the same patient. Available studies have focused on the simultaneous form, whereas there is limited information on sequential PBC-AIH. We carried out a retrospective study of patients who sequentially developed PBC-AIH overlap syndrome.The medical data of 1065 patients diagnosed with PBC (n=483) and AIH (n=582) were retrospectively analyzed.A sequential development of PBC-AIH was observed in 19 (1.8\%) patients after a mean of 6.5 (1-14) years of follow-up. AIH developed in 12 (2.5\%) PBC patients, whereas PBC occurred in seven (1.2\%) patients with AIH. The baseline serologic and histological findings of patients who developed PBC-AIH were similar to those of patients with typical PBC or AIH. Eighteen patients were treated with a combination of ursodeoxycholic acid (UDCA) and immunosuppression after the diagnosis of PBC-AIH was established. One patient showed a spontaneous resolution of hepatitic flare under UDCA therapy. Biochemical remission was achieved in 16 patients, whereas three progressed to decompensated cirrhosis and required liver transplantation.The sequential overlap of PBC-AIH can occur during the follow-up of patients with pure PBC or AIH. In our cohort, we could not identify any factors that predicted the development of this rare condition. The combination of UDCA and immunosuppression seems to be an appropriate therapy in the setting of PBC-AIH.

Sequential presentation of primary biliary cirrhosis and autoimmune hepatitis / C. Efe;E. Ozaslan;A. Heurgué-Berlot;T. Kav;C. Masi;T. Purnak;M. Torgutalp;L. Muratori;S. Bresson-Hadni;G. Thiéfin;T. D. Schiano;P. Muratori;S. Wahlin. - In: EUROPEAN JOURNAL OF GASTROENTEROLOGY & HEPATOLOGY. - ISSN 0954-691X. - STAMPA. - 26:(2014), pp. 532-537. [10.1097/MEG.0000000000000075]

Sequential presentation of primary biliary cirrhosis and autoimmune hepatitis.

MURATORI, LUIGI;MURATORI, PAOLO;
2014

Abstract

Primary biliary cirrhosis (PBC)-autoimmune hepatitis (AIH) overlap syndrome is used to describe the coexistence of both diseases, with either a sequential or a simultaneous presentation in the same patient. Available studies have focused on the simultaneous form, whereas there is limited information on sequential PBC-AIH. We carried out a retrospective study of patients who sequentially developed PBC-AIH overlap syndrome.The medical data of 1065 patients diagnosed with PBC (n=483) and AIH (n=582) were retrospectively analyzed.A sequential development of PBC-AIH was observed in 19 (1.8\%) patients after a mean of 6.5 (1-14) years of follow-up. AIH developed in 12 (2.5\%) PBC patients, whereas PBC occurred in seven (1.2\%) patients with AIH. The baseline serologic and histological findings of patients who developed PBC-AIH were similar to those of patients with typical PBC or AIH. Eighteen patients were treated with a combination of ursodeoxycholic acid (UDCA) and immunosuppression after the diagnosis of PBC-AIH was established. One patient showed a spontaneous resolution of hepatitic flare under UDCA therapy. Biochemical remission was achieved in 16 patients, whereas three progressed to decompensated cirrhosis and required liver transplantation.The sequential overlap of PBC-AIH can occur during the follow-up of patients with pure PBC or AIH. In our cohort, we could not identify any factors that predicted the development of this rare condition. The combination of UDCA and immunosuppression seems to be an appropriate therapy in the setting of PBC-AIH.
2014
Sequential presentation of primary biliary cirrhosis and autoimmune hepatitis / C. Efe;E. Ozaslan;A. Heurgué-Berlot;T. Kav;C. Masi;T. Purnak;M. Torgutalp;L. Muratori;S. Bresson-Hadni;G. Thiéfin;T. D. Schiano;P. Muratori;S. Wahlin. - In: EUROPEAN JOURNAL OF GASTROENTEROLOGY & HEPATOLOGY. - ISSN 0954-691X. - STAMPA. - 26:(2014), pp. 532-537. [10.1097/MEG.0000000000000075]
C. Efe;E. Ozaslan;A. Heurgué-Berlot;T. Kav;C. Masi;T. Purnak;M. Torgutalp;L. Muratori;S. Bresson-Hadni;G. Thiéfin;T. D. Schiano;P. Muratori;S. Wahlin
File in questo prodotto:
Eventuali allegati, non sono esposti

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11585/393060
 Attenzione

Attenzione! I dati visualizzati non sono stati sottoposti a validazione da parte dell'ateneo

Citazioni
  • ???jsp.display-item.citation.pmc??? 6
  • Scopus 22
  • ???jsp.display-item.citation.isi??? 18
social impact