Kimura disease and IgG4-related disease: Same disease spectrum or separate co-existing conditions?

Here we report the case of a previously healthy 42-year old gentleman presenting with lacrimal, parotid and submandibular gland enlargement with associated pulmonary nodules, a pancreatic cyst and radiographic features of pancreatitis. Lacrimal gland biopsy indicated features in keeping with Kimura disease; however the clinical picture was not entirely typical. Prior to commencing treatment a further biopsy was sought from an alternative site, left submandibular gland, which confirmed features of IgG4-related disease. Although Kimura disease and IgG4-related disease share various clinical, serological and histopathological features; they are recognised as two distinct clinical entities. We discuss the possibility that the two conditions are on the same disease spectrum.