Korean J Med.  2015 Dec;89(6):690-694. 10.3904/kjm.2015.89.6.690.

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

• Kim JE
• Cha JJ
• Kim SW
• Hong YA
• Ko GJ
• Kwon YJ

• KMID: 2268926
• DOI: http://doi.org/10.3904/kjm.2015.89.6.690

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.

Keyword

IgG4; Mycophenolic acid; MPA; Eosinophilia

MeSH Terms

Appetite
Cyclophosphamide
Dizziness
Eosinophilia
Humans
Immunoglobulin G
Male
Middle Aged
Mycophenolic Acid*
Pancreatitis
Prednisolone
Steroids
Thyroid Gland
Thyroiditis
Cyclophosphamide
Immunoglobulin G
Mycophenolic Acid
Prednisolone
Steroids