Diagnostic criteria and long-term outcomes in AIH-PBC variant syndrome under combination therapy

Stoelinga, A.E.C.; Biewenga, M.; Drenth, J.P.H.; Verhelst, X.; Meer, A.J.P. van der; Boer, Y.S. de; ... ; Dutch Autoimmune Hepatitis Study (2024)

Article / Letter to editor

Background & Aims: Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) can co-exist in AIH-PBC, requiring combined treatment with immunosuppression and ursodeoxycholic acid (UDCA). The Paris criteria are commonly used to identify these patients; however, the optimal diagnostic criteria are unknown. We aimed to evaluate the use and clinical relevance of both Paris and Zhang criteria. Methods: Eighty-three patients with a clinical suspicion of AIH-PBC who were treated with combination therapy were included. Histology was re-evaluated. Characteristics and long-term outcomes were retrospectively compared to patients with AIH and PBC. Results: Seventeen (24%) patients treated with combination therapy fulfilled the Paris criteria. Fifty-two patients (70%) fulfilled the Zhang criteria. Patients who met Paris and Zhang criteria more often had inflammation and fibrosis on histology compared to patients only meeting the Zhang criteria. Ten-year liver transplant (LT)-free survival was 87.3% (95% CI 78.9–95.7%) in patients with AIH-PBC. This did not differ in patients in or outside the Paris or Zhang criteria (p = 0.46 and p = 0.40, respectively) or from AIH (p = 0.086). LT-free survival was significantly lower in patients with PBC and severe hepatic inflammation – not receiving immunosuppression – compared to those with AIH-PBC (65%; 95% CI 52.2–77.8% vs. 87%; 95% CI 83.2–90.8%; hazard ratio 0.52; p = 0.043). Conclusions: In this study, patients with AIH-PBC outside Paris or Zhang criteria were frequently labeled as having AIH-PBC and were successfully treated with combination therapy with similar outcomes. LT-free survival was worse in patients with PBC and hepatic inflammation than in those treated as having AIH-PBC. More patients may benefit from combination therapy.

• Variant syndrome
• Autoimmune Hepatitis
• Primary Biliary Cholangitis
• ursodeoxycholic acid
• glucocorticoids
• immunosuppression

• All authors: Stoelinga, A.E.C.; Biewenga, M.; Drenth, J.P.H.; Verhelst, X.; Meer, A.J.P. van der; Boer, Y.S. de; Bouma, G.; Vries, E.S. de; Verdonk, R.C.; Berg, A.P. van der; Brouwer, J.T.; Vanwolleghem, T.; Lammers, W.; Beuers, U.; Sarasqueta, A.F.; Verheij, J.; Roskams, T.; Crobach, S.; Tushuizen, M.E.; Hoek, B. van; Dutch Autoimmune Hepatitis Study
• Date: 2024-07-31
• Journal: JHEP Reports
• Volume: 6
• Issue: 7
• DOI: doi:10.1016/j.jhepr.2024.101088