In 1888, Mikulicz-Radecki reported a case exhibiting bilateral, painless and symmetrical swellings of the lacrimal, parotid and submandibular glands. In 1953, Morgan and Castleman suggested that Mikulicz disease (MD) should be reconsidered as one of the manifestations of primary Sjögren's syndrome (pSS) as they shared many histopathological characteristics. But recently, several groups demonstrated that MD may differ from pSS. Actually, MD may partly be an IgG4-related systemic disease, the other components of which are still undelineated and include autoimmune pancreatitis, chronic sclerosing sialoadenitis, Riedel's thyroiditis, some cases of cholangitis, retroperitoneal fibrosis.1 Masaki defined a new clinical entity, the IgG4 multiorgan lymphoproliferative syndrome (MOLPS), which includes these manifestations.2 It is characterised by hyper-IgG4 γ-globulinaemia and IgG4 plasma cell infiltration. There are a number of differences with …