Mikuliczs disease has been considered a part of primary Sjogrens syndrome since Morgans 1953 report. Mikuliczs disease is a unique condition involving enlargement of the lacrimal and salivary glands, as is also seen in Sjogrens syndrome; however, Mikuliczs disease is clinically characterized by infrequent autoimmune reactions and responsiveness to glucocorticoid. Fifty years after Morgans study, the understanding of Mikuliczs disease has changed considerably. We first found elevated levels of serum IgG4 and prominent infiltration by plasmacytes bearing IgG4 in the lacrimal and salivary glands in Mikuliczs disease. These findings do not occur in Sjogrens syndrome. The various extraglandular complications of Mikuliczs disease, including autoimmune pancreatitis, tubulointerstitial nephritis, have gradually been clarified in recent reports. These conditions show similar histopathological findings to Mikuliczs disease, indicating that Mikuliczs disease is a part of a broader entity of systemic IgG4-related disease. We herein describe the clinical features of Mikuliczs disease and its extraglandular lesions.
Keywords: Immunoglobulin G4, Mikulicz's disease, autoimmune pancreatitis, tubulointerstitial nephritis, Sjogren's syndrome, IgG4-related disease, submandibular glands, Enlargement of lacrimal gland, lymphadenopathy, IgG4-producing plasmacytes
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