Belimumab treatment in autoimmune hepatitis and primary biliary cholangitis - a case series.
AIH, autoimmune hepatitis
ALT, alanine aminotransferase
AMA, anti-mitochondrial antibodies
ANA, anti-nuclear antibodies
AP, alkaline phosphatase
APS, anti-phospholipid-antibody syndrome
AZA, azathioprine
Autoimmune hepatitis
Autoimmune liver disease
BDN, budesonide
Belimumab
CI, calcineurin inhibitor
CyA, cyclosporine A
INR, international normalized ratio
IgG, immunoglobulin G
IgM, immunoglobulin M
LC 1, liver cytosol 1 antibodies
LKM-1, liver-kidney-microsomal antibodies
MMF, mycophenolate mofetil
MRCP, magnetic resonance cholangiopancreatography
NA, not applicable
NRH, nodular regenerative hyperplasia
PBC, primary biliary cholangitis
PDN, prednisolone
PLA2R, anti-phospholipase 2 receptor antibody
PSC, primary sclerosing cholangitis
Primary biliary cholangitis
RF, rheumatoid factor
SLA, soluble liver antigen antibodies
SLE, systemic lupus erythematosus
SMA, smooth-muscle cell antibodies
SS-A, SS-A (Ro) antibodies
SS-B, SS-B (La) antibodies
Sjögren's disease
TNFi, tumor necrosis factor inhibitor
UDCA, ursodeoxycholic acid
ULN, upper limit of normal
disease-modifying anti-rheumatic drugs, DMARDs
Journal
Journal of translational autoimmunity
ISSN: 2589-9090
Titre abrégé: J Transl Autoimmun
Pays: Netherlands
ID NLM: 101759413
Informations de publication
Date de publication:
2023
2023
Historique:
received:
08
01
2023
accepted:
12
01
2023
entrez:
31
1
2023
pubmed:
1
2
2023
medline:
1
2
2023
Statut:
epublish
Résumé
The majority of patients with autoimmune hepatitis (AIH) achieve complete remission with established treatment regiments. In patients with intolerance or insufficient response to these drugs, the remaining options are limited and novel treatment approaches necessary. In primary biliary cholangitis (PBC), ursodeoxycholic acid (UDCA) and fibrates have improved prognosis dramatically, but there remains a proportion of patients with refractory disease.In patients with refractory AIH and/or PBC, we used a novel treatment strategy with the To retrospectively investigate treatment response in six patients with AIH or PBC with or without concomitant Sjögren's disease treated with the In all three patients with AIH, belimumab improved disease control and helped by-pass or reduce problematic side effects from corticosteroids and calcineurin inhibitors. In PBC patients (n = 3), there was no clear improvement of liver function tests, despite reduction or normalization of IgM. All patients with concomitant Sjögren's disease (n = 3) had an improvement of sicca symptoms and two out of three patients experienced an initially marked reduction in fatigue, which lessened over time. Belimumab may be a promising treatment option for patients with AIH and further investigations are needed. In PBC however, response was not convincing. The effects on sicca symptoms and fatigue were encouraging.
Sections du résumé
Background
UNASSIGNED
The majority of patients with autoimmune hepatitis (AIH) achieve complete remission with established treatment regiments. In patients with intolerance or insufficient response to these drugs, the remaining options are limited and novel treatment approaches necessary. In primary biliary cholangitis (PBC), ursodeoxycholic acid (UDCA) and fibrates have improved prognosis dramatically, but there remains a proportion of patients with refractory disease.In patients with refractory AIH and/or PBC, we used a novel treatment strategy with the
Aims and methods
UNASSIGNED
To retrospectively investigate treatment response in six patients with AIH or PBC with or without concomitant Sjögren's disease treated with the
Results
UNASSIGNED
In all three patients with AIH, belimumab improved disease control and helped by-pass or reduce problematic side effects from corticosteroids and calcineurin inhibitors. In PBC patients (n = 3), there was no clear improvement of liver function tests, despite reduction or normalization of IgM. All patients with concomitant Sjögren's disease (n = 3) had an improvement of sicca symptoms and two out of three patients experienced an initially marked reduction in fatigue, which lessened over time.
Conclusions
UNASSIGNED
Belimumab may be a promising treatment option for patients with AIH and further investigations are needed. In PBC however, response was not convincing. The effects on sicca symptoms and fatigue were encouraging.
Identifiants
pubmed: 36718275
doi: 10.1016/j.jtauto.2023.100189
pii: S2589-9090(23)00002-3
pmc: PMC9883290
doi:
Types de publication
Journal Article
Langues
eng
Pagination
100189Informations de copyright
© 2023 The Author(s).
Déclaration de conflit d'intérêts
The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
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