IgG4-related sclerosing cholangitis- A great mimicker

Area of Science:

• Gastroenterology
• Immunology
• Pathology

Background:

• Immunoglobulin G4-related disease (IgG4 RD) is a chronic inflammatory condition affecting multiple organs.
• IgG4-related sclerosing cholangitis (IgG4 SC) is the bile duct manifestation of IgG4 RD, frequently linked with autoimmune pancreatitis (AIP).
• IgG4 SC can present with symptoms similar to other biliary diseases, complicating diagnosis.

Purpose of the Study:

• To describe two cases of IgG4-related cholangitis that mimicked primary sclerosing cholangitis.
• To highlight the diagnostic challenges in differentiating IgG4 SC from other forms of sclerosing cholangitis.
• To emphasize the importance of considering IgG4 RD in the differential diagnosis of biliary strictures.

Main Methods:

• Case report of two patients diagnosed with IgG4-related cholangitis.
• Review of clinical presentation, imaging findings, serological markers, and histopathological features.
• Comparison of diagnostic criteria for IgG4 SC and primary sclerosing cholangitis.

Main Results:

• Both patients presented with biliary abnormalities suggestive of primary sclerosing cholangitis.
• Histopathological examination revealed characteristic features of IgG4-related disease, including IgG4-positive plasma cell infiltration and fibrosis.
• Diagnosis of IgG4 SC was confirmed, distinct from primary sclerosing cholangitis.

Conclusions:

• IgG4-related cholangitis can present as a diagnostic mimic of primary sclerosing cholangitis.
• Recognition of IgG4 RD is crucial for accurate diagnosis and appropriate management of biliary disorders.
• Further research is needed to elucidate the pathogenesis and optimize treatment strategies for IgG4 SC.