A case of Sj?gren's syndrome with remarkable swelling of major salivary glands relating to localized amyloidosis

摘要

Sj?gren's syndrome (SS) is well known as an autoimmune disease with destruction of salivary gland tissue and functional disorder. Recently, it was pointed out that SS occurred as a partial lesion of systemic amyloidosis. We report here a case of SS associated with localized amyloidosis which caused marked swelling of bilateral submandibular and parotid glands. Additionally, the relationshiops between SS and systemic or localized amyloidosis is discussed.Sj?gren's syndrome (SS) is characterized by sicca complex in an association with connective tissue disease. On the other hand, it was pointed out that SS occurred as a part of systemic amyloidosis. Amyloidosis is a disorder of protein metabolism in which normally soluble autologous proteins are deposited in tissues as abnormal insoluble fibrils. They are usually characterized by an intra- and extracellular deposition of amyloid in various tissues and are classified as localized or systemic amyloidosis (Gogel HK, 1983). In oral and maxillofacial region, it is uncommon that primary amyloidosis causing xerostomia occurred. This article describes an unusual case presented SS with localized amyloidosis of salivary glands.