A Case of IgG4-related Disease (Mikulicz Disease) that Took Long Time to Make a Diagnosis

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IgG4-related disease (Mikulicz disease) was first reported by Mikulicz in 1892 in a case of a man with bilateral swelling of the lachrymal, parotid, and submandibular glands. However, since Morgan and Castleman examined the pathological features of what they called Mikulicz disease in the 1950s, Mikulicz disease's been considered as a part of Sjögren's syndrome. In 2000, Tsubota et al. reported the difference in salivary gland destruction patterns between Mikulicz disease and Sjögren's syndrome, and explained the clinical differences of each disease. In 2004, Yamamoto et al. reported that the serum IgG4 level is extremely elevated in Mikulicz disease patients. The concept of a systemic IgG4-related syndrome came with the recognition of autoimmune pancreatitis, in which Hamano et al. reported in 2001 that the serum IgG4 level was elevated. Since that time, Mikulicz disease has been considered as a part of a systemic IgG4-related syndrome. We report herein on the case of a man who came to our institute in 2001 with bilateral swelling of the submandibular glands. We initially suspected malignant lymphoma and some other diseases, but during the long course of more investigations and follow-up, we finally diagnosed the condition as IgG4-related disease (Mikulicz disease).