Endoscopic approach for pancreatobiliary involvement in IgG4 related disease

Abstract

Introduction

Immunoglobulin G4-related disease (IgG4-RD) is a rare, systemic autoimmune fibroinflammatory disorder affecting the pancreatobiliary tract, often mimicking malignancies. Diagnosis relies on clinical and histological features, using Mayo Clinic HISORt and Japan Biliary Association criteria.

Case series

Two patients with IgG4-RD presented with abdominal symptoms and abnormal liver function tests. Imaging and endoscopic ultrasound (EUS) revealed bile duct stenosis and pancreatic abnormalities. Elevated serum IgG4 levels and histopathological findings confirmed the diagnosis. Both patients responded well to prednisone, showing clinical improvement and normalization of liver function tests.

Discussion

IgG4-RD represents a diagnostic challenge due to its diverse manifestations. Accurate diagnosis requires a multidisciplinary approach, incorporating clinical, radiological, serological, and histopathological findings. Early recognition and corticosteroid therapy are crucial for achieving remission.

Conclusion

The establishment of diagnostic criteria and advanced imaging techniques has improved the management of IgG4-RD. However, distinguishing it from other biliary disorders remains challenging, necessitating ongoing research and collaboration among medical professionals.