Salivary Gland Lesions in Mikulicz’s Disease and Küttner’s Tumor

Abstract

An extremely diverse spectrum of diseases can cause salivary gland swelling. Disorders termed Mikulicz’s disease and Küttner’s tumor have long been recognized as chronic inflammatory conditions for more than a century and regarded as separate conditions, both associated with painless non-tumorous lesions of the salivary glands. Lacrimal gland swelling is also associated with Mikulicz’s disease, which for more than 50 years was considered erroneously to be a subtype of Sjögren’s syndrome. Developments in recent years have made it clear that although disorders such as Sjögren’s syndrome, sarcoidosis, lymphoma, and others can cause swelling of the salivary and lacrimal glands, Mikulicz’s disease is a distinct subset of IgG4-related disease (IgG4-RD). Many patients previously diagnosed with Küttner’s tumors, or chronic sclerosing submandibular sialadenitis, also in fact have IgG4-RD. These conditions are associated with characteristic histopathological features, increased concentrations of IgG4-bearing plasma cells in affected organs, and often elevated levels of IgG4 in serum. In this chapter, we outline clinical aspects of these two conditions, including their historical background.