Vol.103 No.3 contentsPRACTICA OTO-RHINO-LARYNGOLOGICA
Vol. 103 No. 3 March 2010
A Case of IgG4 Disease
Taketoshi Nogaki, Tomohisa Umiyama and Keiichiro Okuno
(Tokyo Metropolitan Health and Medical Treatment Corporation Ebara Hospital)
Immunoglobulin G4 (IgG4) is thought to be involved in inflammatory IgG4-related disease pathology typically diagnosed clinically as Mikulicz's disease or autoimmune pancreatitis. A recent proposal classifies IgG4-related disease as systemic, and we report a case believed to be IgG4-related disease.
A 76-year-old man was first seen on May 30, 2008, for submental swelling gradually developing since autumn 2007. Physical findings were a dry oral cavity, submental swelling and lymph node swelling. Magnetic resonance imaging (MRI) showed mediastinal lymph node hyperplasia and blood tests increased soluble IL-2 receptors, suggesting malignant lymphoma. Submaxillary gland biopsy on August 8, 2008, showed no clear malignant findings histopatholpgically, but noted chronic sialadenitis. IgG4 immune staining showed IgG4-positive plasmacytes infiltration and blood tests confirmed increased IgG4. Based on these findings, we diagnosed the case as IgG4-related disease, initiating oral prednisolone at 40 mg/day. Submaxillary gland swelling decreased significantly and soluble IL-2 receptors returned to normal. When MRI showed that submaxillary gland and lymph node swelling had been eliminated, prednisolone administration was gradually decreased.
Subjects with IgG4-related disease may have bilateral cervical lymph node swelling and increased soluble IL-2 receptor levels, and this case suggests the importance of considering IgG4-related disease in differential diagnosis.
Key words :Immunoglobulin G4, IgG4-related disease, soluble IL-2 receptor