Table 2.
Characteristics when belimumab was started and stopped, if applicable, as well as results at the last follow-up.
| Timeline | START OF BELIMUMAB |
LAST FOLLOW-UP WITH BELIMUMAB |
LAST FOLLOW-UP |
|
|---|---|---|---|---|
| 01/20 | 10/21 | 11/22 | ||
| Patient1 | ALT(<35 U/L) | 18 | 23 | 259 |
| AP(<104 U/L) | 119 | 122 | 182 | |
| IgG(<16 g/l) | 32.2 | 33.5 | 34.6 | |
| IgM(<2.3 g/l) | 3.02 | 1.92 | 4.82 | |
| Fibroscan(kPa) | 11.2 | 6.2 | 6.2 | |
| Histology | Before belimumab 2019 (already under treatment with 20 mg PDN): florid bile duct lesions, F2 | |||
| Treatment | PDN 7.5 mg UDCA |
Belimumab (21 months) UDCA |
UDCA | |
| Patient 2 | Timeline | 06/21 | 08/22 | |
| ALT(<50 U/L) | 49 | 61 | ||
| AP (<129 U/L) | 152 | 74 | ||
| IgG (<16 g/l) | 8.4 | 8.4 | ||
| IgM (<2.3 g/l) | 1.43 | 1.07 | ||
| Fibroscan (kPa) | 9.0 | 5.9 | ||
| Histology | 2018 (under immunosuppression): no inflammation, cholestatic phenotype of hepatocytes, severe ductopenia (100%), nodular regenerative hyperplasia, F2 | 2021, 4 months after start of belimumab, 1 month after start of bezafibrate and stop of PDN with sudden increase of liver values: periductal mixed inflammatory infiltrate, ductopenia 3/17 portal fields, no florid duct lesions, one microgranuloma, F1 | ||
| Treatment | PDN 10 mg MMF 2 × 1g CyA 100–120 ng/ml UDCA |
PDN 5 mg CyA 100–120 ng/ml Belimumab (14 months) UDCA |
||
| Patient 3 | Timeline | 09/21 | 09/22 | |
| ALT (<50 U/L) | 30 | 27 | ||
| AP (<129 U/L) | 73 | 80 | ||
| IgG (<16 g/l) | 11.4 | 12.4 | ||
| IgM (<2.3 g/l) | 2.24 | 1.88 | ||
| Fibroscan (kPa) | 8.8 | NA | ||
| Histology | NA | NA | ||
| Treatment | PDN 10 mg CyA 100–120 ng/ml |
CyA 75–100 ng/ml Belimumab (8 months) |
||
| Patient 4 | Timeline | 10/20 | 07/22 | |
| ALT (<35 U/L) | 22 | 19 | ||
| AP (<104 U/L) | 126 | 201 | ||
| IgG (<16 g/l) | 14.3 | 11.4 | ||
| IgM (<2.3 g/l) | 2.95 | 1.97 | ||
| Fibroscan (kPa) | 10.1 | 6.1 | ||
| Histology | 2020 before belimumab: portal inflammation, focal interface activity, florid duct lesions, ductopenia 4/10 portal fields, F3 | May 2022 portal inflammation, focal interface activity, ductopenia 6/9 portal fields, F3 | ||
| Treatment | UDCA Bezafibrate |
UDCA Bezafibrate Belimumab (21 months) |
||
| Patient 5 | Timeline | 10/20 | 09/21 | 05/22 |
| ALT (<35 U/L) | 119 | 43 | 58 | |
| AP (<104 U/L) | 183 | 169 | 174 | |
| IgG (<16 g/l) | 14.1 | 9.6 | 13.3 | |
| IgM (<2.3 g/l) | 5.22 | 1.89 | 0.96 | |
| Fibroscan (kPa) | 7.0 | 8.0 | 9.2 | |
| Histology | August 2016 portal mixed infiltrate with plasma cells, one florid duct lesion, ductopenia 9/14 bile ducts, F2 | May 2022 sparse portal infiltrate, no granulomas, no florid duct lesion, ductopenia, F2 | ||
| Treatment | UDCA Fenofibrate |
UDCA Fenofibrate Belimumab (11 months) |
UDCA Fenofibrate Upadacitinib for the RA |
|
| Patient 6 | Timeline | 01/22 | 04/22 | 05/22 |
| ALT (<35 U/L) | 69 | 63 | 51 | |
| AP (<104 U/L) | 126 | 164 | 91 | |
| IgG (<16 g/l) | 15.0 | 14.7 | 14.4 | |
| IgM (<2.3 g/l) | 4.22 | 5.08 | 3.95 | |
| Fibroscan (kPa) | 5.5 | NA | NA | |
| Histology | December 2020 portal inflammation with massive plasma cells, florid bile duct lesions, ductopenia 4/10 portal fields, F2 | April 2022 portal inflammation with some interphase activity and single hepatocellular necroapoptosis, some florid duct lesions, ductopenia 9/11 portal fields, F3 | ||
| Treatment | UDCA Bezafibrate |
UDCA Bezafibrate Belimumab (4 weeks) |
UDCA Bezafibrate |