Long-term follow-up of children and young adults with autoimmune hepatitis treated with cyclosporine.
Adolescent
Azathioprine
/ therapeutic use
Child
Child, Preschool
Cholangitis, Sclerosing
/ complications
Cyclosporine
/ administration & dosage
Drug Therapy, Combination
Female
Follow-Up Studies
Hepatitis, Autoimmune
/ complications
Humans
Immunosuppressive Agents
/ administration & dosage
Liver Transplantation
Male
Mycophenolic Acid
/ therapeutic use
Prednisone
/ therapeutic use
Remission Induction
Retrospective Studies
Syndrome
Time Factors
Treatment Outcome
Autoimmune hepatitis
Autoimmune sclerosing cholangitis
Cyclosporine
Glomerular filtration rate
Overlap syndrome
Journal
Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver
ISSN: 1878-3562
Titre abrégé: Dig Liver Dis
Pays: Netherlands
ID NLM: 100958385
Informations de publication
Date de publication:
05 2019
05 2019
Historique:
received:
13
02
2018
revised:
22
10
2018
accepted:
24
10
2018
pubmed:
7
12
2018
medline:
6
2
2020
entrez:
4
12
2018
Statut:
ppublish
Résumé
Cyclosporine (CSA) is an alternative treatment for autoimmune hepatitis (AIH), however, its unknown long-term safety and efficacy have limited its use. Examine the long-term outcome of children and young adults with AIH treated with CSA for at least 4 years. Twenty patients were included in this retrospective study: 15 with classical AIH and 5 with autoimmune hepatitis/autoimmune sclerosing cholangitis overlap syndrome (ASC). CSA was administered as first (12 patients) or second-line (8 patients) treatment, alone or in combination with azathioprine or mycophenolate mofetil and/or prednisone. CSA determined initial clinical and biochemical remission in all patients. At the end of follow-up (median 8.6; range 4-20.4 years), all patients are alive with their native liver; 15 in complete remission (75%), 2 with incomplete response to treatment and 3 listed for liver transplant. Side effects were mild and transitory after dose tapering or, in 1 case, after CSA withdrawal. Hypertrichosis and moderate gingival hyperplasia were the most frequent. Two patients presented mild transient glomerular filtration rate (GFR) reduction. Median GFR at the beginning and end of treatment was not statistically different for all patients. CSA was effective and safe in the long-term treatment of our cohort of patients with AIH, tailoring the treatment remains key-points during CSA administration.
Sections du résumé
BACKGROUND
Cyclosporine (CSA) is an alternative treatment for autoimmune hepatitis (AIH), however, its unknown long-term safety and efficacy have limited its use.
AIMS
Examine the long-term outcome of children and young adults with AIH treated with CSA for at least 4 years.
METHODS
Twenty patients were included in this retrospective study: 15 with classical AIH and 5 with autoimmune hepatitis/autoimmune sclerosing cholangitis overlap syndrome (ASC). CSA was administered as first (12 patients) or second-line (8 patients) treatment, alone or in combination with azathioprine or mycophenolate mofetil and/or prednisone.
RESULTS
CSA determined initial clinical and biochemical remission in all patients. At the end of follow-up (median 8.6; range 4-20.4 years), all patients are alive with their native liver; 15 in complete remission (75%), 2 with incomplete response to treatment and 3 listed for liver transplant. Side effects were mild and transitory after dose tapering or, in 1 case, after CSA withdrawal. Hypertrichosis and moderate gingival hyperplasia were the most frequent. Two patients presented mild transient glomerular filtration rate (GFR) reduction. Median GFR at the beginning and end of treatment was not statistically different for all patients.
CONCLUSIONS
CSA was effective and safe in the long-term treatment of our cohort of patients with AIH, tailoring the treatment remains key-points during CSA administration.
Identifiants
pubmed: 30502231
pii: S1590-8658(18)31214-3
doi: 10.1016/j.dld.2018.10.018
pii:
doi:
Substances chimiques
Immunosuppressive Agents
0
Cyclosporine
83HN0GTJ6D
Mycophenolic Acid
HU9DX48N0T
Azathioprine
MRK240IY2L
Prednisone
VB0R961HZT
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
712-718Informations de copyright
Copyright © 2018 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.