IgG4-related dacryoadenitis and sialadenitis

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Disease Overview

IgG4-related dacryoadenitis and sialoadenitis (formerly called Mikulicz disease) is an IgG4-related disease characterized by inflammation of the lacrimal glands (which produce tears), parotid glands, and submandibular glands (two of the major salivary glands).[13820] In some cases, it also affects other glands or organs.[13820][13824] The condition is usually painless, mainly causing mouth and eye dryness, and swelling over the affected glands.[13820][13824] When other organs are affected, it can be accompanied by complications such as autoimmune pancreatitis, retroperitoneal fibrosis, and tubulointerstitial nephritis.[13820] The underlying cause of IgG4-related disease is still not known.[13822] Treatment involves corticosteroids, which are usually effective.[13823][13824] Medicines that suppress the immune system (immunosuppressants) may also be used in cases that do not respond to corticosteroids.[13823][13824]

IgG4-related dacryoadenitis and sialoadenitis was previously considered a subtype of Sjogren syndrome, but it is now known to be a distinct condition.[13824]


Synonyms

  • Mikulicz's disease (former)
  • Mikulicz disease (former)
  • Mikulicz syndrome (former)
  • Chronic dacryoadenitis and sialadenitis

For more information, visit GARD.

National Organization for Rare Disorders