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Nihon Jibiinkoka Gakkai Kaiho. 2010 Oct;113(10):798-804.

[IgG4-related Mikulicz's disease: a report of 3 cases].

[Article in Japanese]

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  • 1Department of Otolaryngology, Saitama Medical University, Saitama.



We report three cases of hyper-IgG4 disease with synchronous or asynchronous lymphocytic infiltration onset, IgG4 positive plasma cell infiltration, and fibril formation in multiple exocrine glands and extranodal organs. IgG4-related sialadenitis attracting recent attention has yet to be clarified as a clinical entity.


Case 1, a 61-year-old man, had a submandibular gland sample showing IgG4-positive plasma cell infiltration. Case 2, a 61-year-old man, was diagnosed with IgG4-related Mikulicz's disease confirmed by a sublingual gland sample. Case 3, a 57-year-old woman, had a diagnosis of IgG4-related Mikulicz's disease confirmed based on labial and sublingual gland samples. All reported oral dryness and bilateral submandibular swelling. Cases 1 and 2 recovered following Predonine administration tapered from 30 or 20 mg.


IgG4-related Mikulicz's disease must be recognized as a clinical entity, together with its diagnostic criteria and treatment. Sublingual gland biopsy should be done to confirm its diagnosis following sublingual gland swelling.

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