IgG4-related Disease Controlled by a Low-dose Steroid and Mycophenolic Acid / 대한내과학회지

ABSTRACT

IgG4-related disease (IgG4-RD) is a group of fibro-inflammatory diseases affecting multiple organ systems, pathologically characterized by tissue infiltration with abundant IgG4-positive cells. We here report a case of IgG4-RD that was successfully controlled by mycophenolic acid (MPA). A 62-year-old male visited complaining of dizziness and loss of appetite. He had a clinical history of autoimmune pancreatitis treated with steroids and cyclophosphamide 5 years prior, and had also been diagnosed with Hashimoto's thyroiditis at the age of 55 years. We initially prescribed prednisolone (1 mg/kg), tapered to 5 mg/day over 8 months. However, his eosinophilia gradually deteriorated, and we added cyclophosphamide (1 mg/kg daily) for the next 7 months. His laboratory data fluctuated when he was on low-dose predinisolone and cyclophosphamide, and we thus changed his medication to prednisolone combined with MPA for the following 63 months. Currently, his laboratory findings are stable.