Abstract
This review describes methods to diagnose and treat patients with IgG4-related disease. A diagnosis of IgG4-related disease is based on elevated serum IgG4 concentrations and an increased number of IgG4-positive plasma cells. Differentiating IgG4-related disease from other disorders, especially malignancy, is important. Consensus treatment in Japan consists of an initial dose of prednisolone 0.5mg-0.6mg/kg/day, followed by careful and gradual dose reduction. The cooperation of otolaryngologists and internists is important in the diagnosis and treatment of IgG4-related disease, because the concomitant occurrence head and neck lesions frequently occurs.