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Overlap Syndromes and Associated Immunoglobulin-G4-Related Liver Diseases
Sayan Malakar1, Sanjit Kumar2, Akash Roy3
1Department of Hepatology, Punjab Institute of Liver and Biliary Sciences, Mohali, Punjab, India.
Journal of Clinical and Experimental Hepatology
|June 29, 2026
Summary
Overlap syndromes (OS) present with mixed autoimmune liver disease features. Early diagnosis and management of OS and IgG4-related diseases are crucial for improved patient outcomes and survival.
Area of Science:
- Hepatology
- Immunology
- Gastroenterology
Background:
- Overlap syndromes (OS) represent mixed hepatitic-cholestatic autoimmune liver diseases (AILD).
- These conditions share features of autoimmune hepatitis (AIH), bile duct injury, and autoantibodies associated with primary biliary cholangitis (PBC) or primary sclerosing cholangitis (PSC).
- Increasing recognition of immunoglobulin G4-related disease (IgG4-RD) has led to the identification of IgG4-hepatopathy, IgG4-AIH, and PSC with elevated serum IgG4, often presenting similarly to OS.
Purpose of the Study:
- To review and formulate diagnostic and management strategies for OS based on current evidence.
- To explore the complex relationship between IgG4-related disease and AILD.
- To highlight the importance of early recognition and intervention in OS and related cholestatic disorders.
Main Methods:
- Literature review of current evidence on OS, AILD, and IgG4-RD.
- Analysis of diagnostic criteria and treatment approaches for these conditions.
- Synthesis of information regarding the clinical presentation and prognostic factors.
Main Results:
- Patients with IgG4-related disorders frequently present with OS-like symptoms.
- High serum IgG4 levels in PSC correlate with poorer overall survival.
- Distinguishing between IgG4-AIH and IgG4-hepatopathy may impact treatment and prognosis.
Conclusions:
- Early identification and management of OS and associated cholestatic diseases can enhance patient outcomes and transplant-free survival.
- Understanding the interplay between IgG4-RD and AILD is vital for effective patient care.
- Evidence-based strategies are needed for the diagnosis and management of OS.
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