Primary Biliary Cholangitis (PBC)-Autoimmune Hepatitis (AIH) Variant Syndrome: Clinical Features, Response to Therapy and Long-Term Outcome
Introduction: Standardization of diagnostic criteria of autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) variant syndrome (AIH-PBC VS) has not been achieved so far and evidence-based recommendations for monitoring and treatment of the disease are still lacking. Our study aimed to ass...
| Main Authors: | , , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2023-11-01
|
| Series: | Journal of Clinical Medicine |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2077-0383/12/22/7047 |
| _version_ | 1797458839648337920 |
|---|---|
| author | Markus Graf Christian M. Lange Mona M. Langer Jörn M. Schattenberg Jessica Seessle Julia Dietz Annika Vermehren Florian A. Michael Antonia Mondorf Stefan Zeuzem Anita Pathil Christiana Graf |
| author_facet | Markus Graf Christian M. Lange Mona M. Langer Jörn M. Schattenberg Jessica Seessle Julia Dietz Annika Vermehren Florian A. Michael Antonia Mondorf Stefan Zeuzem Anita Pathil Christiana Graf |
| author_sort | Markus Graf |
| collection | DOAJ |
| description | Introduction: Standardization of diagnostic criteria of autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) variant syndrome (AIH-PBC VS) has not been achieved so far and evidence-based recommendations for monitoring and treatment of the disease are still lacking. Our study aimed to assess the prevalence, biochemical, and serological features, as well as the clinical course, of VS. Methods: We performed a retrospective study including all patients with VS between 1999 and 2020 in four German centers. Data on demographic parameters, biochemical and serological tests, treatment, and outcome were collected. Results: Of 90 patients (3.1%) meeting Paris criteria for VS diagnosis, 65.6% showed AIH and PBC histological features, while biochemical Paris criteria were observed comparatively rarely. Further antibodies, which were not part of the diagnostic criteria of VS, were found in a subgroup of patients with available data (ACA: 30.0%; anti-CENP-A: 25.0%; anti-CENP-B: 33.3%; anti-SP100: 21.4%). Biochemical response was more frequently observed in patients treated with a combined therapy of ursodeoxycholic acid (UDCA) and immunosuppression (IS). Liver cirrhosis was detected in 31 patients (34.4%) and 25 patients (27.8%) developed clinical manifestations of portal hypertension. Conclusions: Biochemical Paris criteria of VS were rarely detected, thus implying that these cut-off values should be redefined. Regarding pharmacological treatment, combined therapy of UDCA and IS appeared to be more effective than monotherapy with UDCA. |
| first_indexed | 2024-03-09T16:42:55Z |
| format | Article |
| id | doaj.art-53e4c88ee7dc4d5d937baf3ec61f19cb |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-09T16:42:55Z |
| publishDate | 2023-11-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-53e4c88ee7dc4d5d937baf3ec61f19cb2023-11-24T14:49:16ZengMDPI AGJournal of Clinical Medicine2077-03832023-11-011222704710.3390/jcm12227047Primary Biliary Cholangitis (PBC)-Autoimmune Hepatitis (AIH) Variant Syndrome: Clinical Features, Response to Therapy and Long-Term OutcomeMarkus Graf0Christian M. Lange1Mona M. Langer2Jörn M. Schattenberg3Jessica Seessle4Julia Dietz5Annika Vermehren6Florian A. Michael7Antonia Mondorf8Stefan Zeuzem9Anita Pathil10Christiana Graf11Department of Internal Medicine I, University Hospital Frankfurt, 60596 Frankfurt am Main, GermanyDepartment of Internal Medicine II, University Hospital Munich, 81377 Munich, GermanyDepartment of Internal Medicine II, University Hospital Munich, 81377 Munich, GermanyDepartment of Internal Medicine I, University Medical Center Mainz, 55131 Mainz, GermanyDepartment of Internal Medicine IV, University of Heidelberg, 69120 Heidelberg, GermanyDepartment of Internal Medicine I, University Hospital Frankfurt, 60596 Frankfurt am Main, GermanyDepartment of Internal Medicine I, University Hospital Frankfurt, 60596 Frankfurt am Main, GermanyDepartment of Internal Medicine I, University Hospital Frankfurt, 60596 Frankfurt am Main, GermanyDepartment of Internal Medicine I, University Hospital Frankfurt, 60596 Frankfurt am Main, GermanyDepartment of Internal Medicine I, University Hospital Frankfurt, 60596 Frankfurt am Main, GermanyDepartment of Internal Medicine I, University Hospital Frankfurt, 60596 Frankfurt am Main, GermanyDepartment of Internal Medicine I, University Hospital Frankfurt, 60596 Frankfurt am Main, GermanyIntroduction: Standardization of diagnostic criteria of autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) variant syndrome (AIH-PBC VS) has not been achieved so far and evidence-based recommendations for monitoring and treatment of the disease are still lacking. Our study aimed to assess the prevalence, biochemical, and serological features, as well as the clinical course, of VS. Methods: We performed a retrospective study including all patients with VS between 1999 and 2020 in four German centers. Data on demographic parameters, biochemical and serological tests, treatment, and outcome were collected. Results: Of 90 patients (3.1%) meeting Paris criteria for VS diagnosis, 65.6% showed AIH and PBC histological features, while biochemical Paris criteria were observed comparatively rarely. Further antibodies, which were not part of the diagnostic criteria of VS, were found in a subgroup of patients with available data (ACA: 30.0%; anti-CENP-A: 25.0%; anti-CENP-B: 33.3%; anti-SP100: 21.4%). Biochemical response was more frequently observed in patients treated with a combined therapy of ursodeoxycholic acid (UDCA) and immunosuppression (IS). Liver cirrhosis was detected in 31 patients (34.4%) and 25 patients (27.8%) developed clinical manifestations of portal hypertension. Conclusions: Biochemical Paris criteria of VS were rarely detected, thus implying that these cut-off values should be redefined. Regarding pharmacological treatment, combined therapy of UDCA and IS appeared to be more effective than monotherapy with UDCA.https://www.mdpi.com/2077-0383/12/22/7047autoimmune hepatitis (AIH)primary biliary cholangitis (PBC)variant syndrome (VS)Paris criteriaimmunosuppressionliver cirrhosis |
| spellingShingle | Markus Graf Christian M. Lange Mona M. Langer Jörn M. Schattenberg Jessica Seessle Julia Dietz Annika Vermehren Florian A. Michael Antonia Mondorf Stefan Zeuzem Anita Pathil Christiana Graf Primary Biliary Cholangitis (PBC)-Autoimmune Hepatitis (AIH) Variant Syndrome: Clinical Features, Response to Therapy and Long-Term Outcome Journal of Clinical Medicine autoimmune hepatitis (AIH) primary biliary cholangitis (PBC) variant syndrome (VS) Paris criteria immunosuppression liver cirrhosis |
| title | Primary Biliary Cholangitis (PBC)-Autoimmune Hepatitis (AIH) Variant Syndrome: Clinical Features, Response to Therapy and Long-Term Outcome |
| title_full | Primary Biliary Cholangitis (PBC)-Autoimmune Hepatitis (AIH) Variant Syndrome: Clinical Features, Response to Therapy and Long-Term Outcome |
| title_fullStr | Primary Biliary Cholangitis (PBC)-Autoimmune Hepatitis (AIH) Variant Syndrome: Clinical Features, Response to Therapy and Long-Term Outcome |
| title_full_unstemmed | Primary Biliary Cholangitis (PBC)-Autoimmune Hepatitis (AIH) Variant Syndrome: Clinical Features, Response to Therapy and Long-Term Outcome |
| title_short | Primary Biliary Cholangitis (PBC)-Autoimmune Hepatitis (AIH) Variant Syndrome: Clinical Features, Response to Therapy and Long-Term Outcome |
| title_sort | primary biliary cholangitis pbc autoimmune hepatitis aih variant syndrome clinical features response to therapy and long term outcome |
| topic | autoimmune hepatitis (AIH) primary biliary cholangitis (PBC) variant syndrome (VS) Paris criteria immunosuppression liver cirrhosis |
| url | https://www.mdpi.com/2077-0383/12/22/7047 |
| work_keys_str_mv | AT markusgraf primarybiliarycholangitispbcautoimmunehepatitisaihvariantsyndromeclinicalfeaturesresponsetotherapyandlongtermoutcome AT christianmlange primarybiliarycholangitispbcautoimmunehepatitisaihvariantsyndromeclinicalfeaturesresponsetotherapyandlongtermoutcome AT monamlanger primarybiliarycholangitispbcautoimmunehepatitisaihvariantsyndromeclinicalfeaturesresponsetotherapyandlongtermoutcome AT jornmschattenberg primarybiliarycholangitispbcautoimmunehepatitisaihvariantsyndromeclinicalfeaturesresponsetotherapyandlongtermoutcome AT jessicaseessle primarybiliarycholangitispbcautoimmunehepatitisaihvariantsyndromeclinicalfeaturesresponsetotherapyandlongtermoutcome AT juliadietz primarybiliarycholangitispbcautoimmunehepatitisaihvariantsyndromeclinicalfeaturesresponsetotherapyandlongtermoutcome AT annikavermehren primarybiliarycholangitispbcautoimmunehepatitisaihvariantsyndromeclinicalfeaturesresponsetotherapyandlongtermoutcome AT florianamichael primarybiliarycholangitispbcautoimmunehepatitisaihvariantsyndromeclinicalfeaturesresponsetotherapyandlongtermoutcome AT antoniamondorf primarybiliarycholangitispbcautoimmunehepatitisaihvariantsyndromeclinicalfeaturesresponsetotherapyandlongtermoutcome AT stefanzeuzem primarybiliarycholangitispbcautoimmunehepatitisaihvariantsyndromeclinicalfeaturesresponsetotherapyandlongtermoutcome AT anitapathil primarybiliarycholangitispbcautoimmunehepatitisaihvariantsyndromeclinicalfeaturesresponsetotherapyandlongtermoutcome AT christianagraf primarybiliarycholangitispbcautoimmunehepatitisaihvariantsyndromeclinicalfeaturesresponsetotherapyandlongtermoutcome |