Belimumab in Autoimmune Liver Diseases with associated Sjögren’s Syndrome

Kolev, Mirjam; Sarbu, Adela-Cristina; Walder, Anna; Moeller, Burkhard; Maurer, Britta; Kollert, Florian; Semmo, Nasser (2 September 2021). Belimumab in Autoimmune Liver Diseases with associated Sjögren’s Syndrome. Swiss medical weekly, 151(S252), p. 16. EMH Media

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Background: Autoimmune hepatitis (AIH) is an auto-inflammatory disease
of the liver with, if untreated, a high mortality rate. About 75% of
patients are responsive to synthetic disease modifying drugs
(sDMARD). Primary biliary cholangitis (PBC) is an inflammatory disease
of small and medium sized bile ducts. Despite standard treatments (ursodeoxycholic
acid (UDCA), fibrates and obeticholic acid (OCA)), a significant
proportion of patients has progressive disease. Twenty percent of
PBC patients have Sjögren’s syndrome (SjS). PBC has many features in
common with SjS: epidemiology, epithelitis, well-characterized autoantibodies
and a poor response to immunosuppressive treatments.
Hypothesis: Based on the increased B cell-activating factor (BAFF) levels
in AIH, PBC and SjS patients and the similarities between PBC and
SjS, we hypothesized that belimumab is effective in AIH and PBC.
Methods: Retrospective analysis of treatment responses to belimumab
in three female patients with AIH and/or PBC with moderate to advanced
liver fibrosis and concomitant SjS. Patient 1: 52y, with AIH, PBC and SjS.
Indication: active AIH with intolerance to previous treatments (AZA,
MMF, rituximab); belimumab since 01/20. Patient 2: 72y, with PBC and
SjS. Indication: refractory PBC despite UDCA and fibrates (OCA declinedby health insurance); belimumab since 11/20. Patient 3: 54y, with PBC
(with ductopenia), SjS and erosive rheumatoid arthritis (RA). RA responding
insufficiently to all commonly used sDMARDs and biologicals. She
was on low dose steroids, HCQ and etanercept. Her PBC was active
despite UDCA and fibrates (OCA not tolerated). Indication: refractory
PBC; belimumab since 11/20. We discontinued etanercept, when
belimumab was started.
Results: Patient 1: Remission of AIH under belimumab. Patient 2: Remission
of PBC after 6 months of belimumab. Patient 3: Stable cholestasis
parameters. Improvement of slightly elevated transaminases and
almost normalization of IgM.
Improvement of sicca symptoms in all patients. Two patients had a transient
improvement in fatigue. RA in patient 3 remained on a level of
moderate disease activity.
Conclusions: These preliminary findings suggest belimumab as a promising
treatment option in AIH and PBC, with so far no safety concerns.
Our study shows how the basket of autoimmune diseases can guide us
to evaluate new drug candidates in a more efficient way and highlights
the strengths of a tight collaboration between hepatology and rheumatology.

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