Belimumab treatment in autoimmune hepatitis and primary biliary cholangitis - a case series.

Kolev, Mirjam; Sarbu, Adela-Cristina; Möller, Burkhard; Maurer, Britta; Kollert, Florian; Semmo, Nasser (2023). Belimumab treatment in autoimmune hepatitis and primary biliary cholangitis - a case series. Journal of translational autoimmunity, 6(100189), p. 100189. Elsevier 10.1016/j.jtauto.2023.100189

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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 anti-B cell activating factor, belimumab. The first three patients had concomitant Sjögren's disease. The connecting element between all three diseases is B cell activation, including elevated levels of the B cell activating factor (BAFF). Furthermore, belimumab has been shown to be beneficial in Sjögren's disease.


To retrospectively investigate treatment response in six patients with AIH or PBC with or without concomitant Sjögren's disease treated with the anti-BAFF therapy belimumab at the University Hospital in Bern, Switzerland.


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.

Item Type:

Journal Article (Original Article)


04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Rheumatology, Clinical Immunology and Allergology
04 Faculty of Medicine > Department of Gastro-intestinal, Liver and Lung Disorders (DMLL) > Clinic of Visceral Surgery and Medicine > Hepatology

Graduate School:

Graduate School for Health Sciences (GHS)

UniBE Contributor:

Kolev, Mirjam, Sarbu, Adela-Cristina, Möller, Burkhard, Maurer, Britta, Kollert, Florian Kim, Semmo, Nasser


600 Technology > 610 Medicine & health








Pubmed Import

Date Deposited:

01 Feb 2023 10:15

Last Modified:

05 Feb 2023 02:26

Publisher DOI:


PubMed ID:


Uncontrolled Keywords:

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




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