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Immunoglobulin (IgG4) sclerosing cholecystitis-camouflaging gall bladder cancer: A case report

Rashpal Singh*, Puneet Mahajan, Rizul Prasher, Vivek Rajdev, Jagwinder Singh, Cliffin Mathai Kattoor, Kavita Mardi, Jagdish Gupta

Background: IgG4 related disease is a rare systemic disorder having an underlying autoimmune cause. These disorders mainly affects pancreatico biliary tree pancreas, gall bladder and biliary tree (extra hepatic as well intrahepatic), but can also affect other part of body. Majority of disorders involving biliary tree are associated with autoimmune pancreatitis component. These disorders are difficult to diagnose clinically as they can mimic inflammatory as well malignancy and poses a real diagnostic challenge to manage and treat.

Case Presentation: 64 years old female known diabetic evaluated for pain in right hypochondrium. Gall bladder cancer was suspected clinically as well on radiologically basis. Patient underwent extended cholecystectomy as it was a resectable disease. Final histopathology revealed immunoglobulin G4 (IgG4) related cholecystitis which was confirmed after immunohistochemistry for CD 138 and IgG4.This disease could be managed conservatively by giving oral steroids, if it has been picked up preoperatively and major surgical intervention have been avoided. No defined blood test or tumour markers are currently available to diagnose this entity except serum immunoglobulin G4 which is costly and not feasible to get done in all patients especially in developing nations like India.

Conclusion: IgG4 cholecystitis is an immune mediated disease whose pathophysiology is still not completely understood. Every clinician should keep possibility of IgG4 cholecystitis in mind whenever any patient with abnormal gall bladder thickening or gall bladder mass is encountered in their clinical practice, as both these entities have completely different options of treatment. We should not rely solely upon clinical and radiological picture.