Original Article Autoimmune Hepatitis Overlap Syndrome: Clinical Presentation, Treatment Response and Disease Evolution: Tertiary Centre Experience
by Abduljaleel Al Alwan1,2,3*, Ibrahim Altraif1,4, Hamdan Alghamdi1,2,3, Abdulrahman Aljumah5, Tariq Aldebasi1,2,3, Mohammed Omar Amer6, Dua Omar Binjabal7, Ali H. Albenmousa8, Mohammad S. Shawaqfeh3,9, Nataleen A. Albekairy1,3, Lolwa Fetyani3,9, Abdelhameed H. Elfadol3,9, Wesam S. Abdel-Razaq3,9, Abdulkareem M. Albekairy2,3,9, Faisal M. Sanai10,11
1College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia.
2King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia.
3King Abdullah International Medical Research Centre, Ministry of National Guard Health
Affairs, Riyadh, Saudi Arabia.
4Department of Medicine, Suliman Fakeeh Hospital, Riyadh, Saudi Arabia
5College of Medicine, Dar Al Uloom University, Riyadh, Saudi Arabia
6National Liver Institute, Menoufia University, Egypt.
7International Medical Center, Jeddah, Saudi Arabia.
8Organ Transplant Center, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
9College of Pharmacy, King Saud bin Abdulaziz University for Health Sciences, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia.
10Gastroenterology Section, Department of Medicine, King Abdulaziz Medical City, King Abdullah International Medical Research Centre, Ministry of National Guard Health
Affairs, Jeddah, Saudi Arabia
11Liver Disease Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia
*Corresponding author: Abduljaleel Al Alwan, Department of Hepatobiliary Sciences and Organs Transplant Center, King Abdulaziz Medical City, Ministry of National Guard Health Affairs, P.O. Box 22490, Riyadh 11426, Saudi Arabia.
Received Date: 20 August 2025
Accepted Date: 01 September 2025
Published Date: 04 September 2025
Citation: Al Alwan A, Altraif I, Alghamdi H, Aljumah A, Aldebasi T, et al. (2025) Autoimmune Hepatitis Overlap Syndrome: Clinical Presentation, Treatment Response and Disease Evolution: Tertiary Centre Experience. J Dig Dis Hepatol 10: 229. https://doi.org/10.29011/2574-3511.100229
Abstract
Background: Autoimmune hepatitis (AIH) overlap syndromes are conditions wherein features of AIH coexist with those of primary sclerosing cholangitis (PSC) or primary biliary cholangitis (PBC). We aimed to evaluate their clinical presentation, treatment response, and disease outcome in an Arab cohort. Methods: AIH overlap patients, based on the “Paris criteria” for PBC/AIH overlap (n=19) and revised IAIHG score for PSC/AIH overlap (n=17) were reviewed at a single center in Saudi Arabia, from 2001 to 2024. Biopsy-proven standalone AIH patients (n=93) were included as controls. The primary endpoint was disease progression, liver transplantation or mortality. Results: Overall, the AIH and overlap syndrome cohorts were diagnosed at similar ages. Post-treatment MELD and CTP scores did not improve in the PBC/AIH cohort (p>0.05), while in PSC/AIH, the CTP (p=0.005) and Mayo Clinic scores improved (p=0.002). In PBC/AIH and PSC/AIH cohorts, disease progression and disease remission rates were comparable. The median relapse-free survival (RFS) was 10.4 years for PBC/AIH, and 13.8 years for PSC/AIH cohorts (p=0.256). The 5- and 10-year RFS rates for the AIH, PBC/AIH, and PSC/AIH cohorts were 92.3% and 72.7%, 81.3% and 53.4%, and 81.4% and 67.9%, respectively. Higher initial Mayo (p=0.035) and CTP score (p=0.028) predicted development of disease composite endpoint (disease progression, liver transplantation or death).Conclusion: Despite lack of standardized treatment protocols, AIH and overlap syndromes respond comparably well to treatment, in addition to similar disease remission and RFS between PBC/AIH and PSC/AIH patients. More extended follow-up studies are warranted for patients with overlap syndrome.
Keywords: Autoimmune Hepatitis; Overlap Syndrome; Primary Sclerosing Cholangitis; Primary Biliary Cholangitis; Treatment; Progression; Survival.
Abbreviations
AIH: Autoimmune Hepatitis
IAIHG: International Autoimmune Hepatitis Group
ALP: Alkaline Phosphatase
ALT: Alanine Transaminase
AMA: Antimitochondrial Antibody
ANA: Antinuclear Antibody
CTP: Child-Turcotte-Pugh
GGT: Gamma-Glutamyl Transferase
IgG: Immunoglobulin G
MELD: Model For End-Stage Liver Disease
PBC: Primary Biliary Cholangitis
PSC: Primary Sclerosing Cholangitis
SMA: Smooth Muscle Antibody
ULN: Upper Limit of Normal Range
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