Immunoglobulin G4-related disease involving multiple systems: A case report

IgG4-related disease (IgG4-RD), an immune-mediated chronic progressive fibroinflammatory disease, can affect the functions of several organs. Some common characteristics can be observed in different IgG4-RDs, such as higher prevalence in middle-aged and elderly male patients, raised serum IgG4 levels, abundant infiltration of IgG4-positive cells and fibrosis, diffuse or localized swelling of the affected organs, and good response to glucocorticoids treatment.

A 72-year-old man complained of left upper abdominal pain 3 mo ago, and he was diagnosed with acute onset of chronic cholecystitis and acute pancreatitis in the local hospital. Pain improved after relevant treatment. Several days ago, his abdominal pain worsened, and he was admitted to our hospital for further treatment. Doppler ultrasound showed that the pancreas presented with sausage-like swelling and the parenchymal echo was diffusely reduced. Gallbladder volume was increased, while the wall was rough and thickened with bilateral signs. Furthermore, the left submandibular gland was enlarged, accompanied with significantly increased blood flow signals. Finally, we found that the adventitia of the abdominal aorta and right iliac artery was thickened locally. Serum IgG4 was elevated to 12600 mg/L. Therefore, the patient was diagnosed with IgG4-RD. After treatment with methylprednisolone, he had an uneventful course and was discharged in good condition.

IgG4-RD can involve almost any organs. Ultrasound has a significant role in timely and accurately diagnosis.