2010 Volume 126 Pages 68-72
Over the years, Mikulicz’s disease has been considered a subtype of Sjögren’s syndrome; but recent studies point out the involvement of IgG4, and this new concept classifies Mikulicz’s disease as a systemic IgG4-related plasmacytic disease, different from Sjögren’s syndrome. We experienced 2 cases (a 53 year old female and a 51 year old male) who presented with swollen lacrimal glands and submandibular glands. Their IgG4 concentrations in the serum were shown to be elevated and prominent IgG4 positive plasma cells were revealed on histopathological examinations. Since other lymphopoliferative diseases were ruled out, from these findings we diagnosed the patients as having Mikulicz’s disease. Treatment with systemic steroids proved effective and the patients showed no other systemic complications. Reports state that in the later stages, other complications such as autoimmune pancreatitis may occur, therefore regular follow up studies are essential.