A case of IgG4-related disease founded from sclerosing sialadenitis

Abstract IgG4-related disease, a new disease entity proposed in Japan, is characterized by the chronic inflammation of various organs, enlargement of affected organs, and reversible hypoadenia. Here, a case of a condition suspected to be IgG4-related sclerosing sialadenitis is reported. A 58-year-old woman consulted us with a primary symptom of bilateral, painless, and submandibular swelling. While salivary gland MRI revealed the symmetric enlargement of the lacrimal and salivary glands, no mass formation was noted. Also, while autoantibody was negative, a diagnosis of IgG4-related disease was made because of a high serum IgG4 level and fibrosis accompanied by IgG4-positive plasma cell infiltration demonstrated by sublingual gland biopsy. Subsequent examinations of the whole body also disclosed autoimmune pancreatitis. Enlargement of the salivary glands rapidly reversed, and pancreatomegaly also gradually regressed, after the beginning of steroid therapy. Since IgG4-related disease is systemic, it is necessary to examine various organs and prevent the progression of their impairment. This disease remains unclear in many respects, and multidisciplinary cooperation is considered important.