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Clinical characteristics of Mikulicz’s disease as an IgG4-related disease

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Abstract

Objectives

Mikulicz’s disease (MD) was considered to be a subtype of Sjögren’s syndrome (SS), based on histopathological similarities. However, recent studies have indicated that patients with MD show high serum IgG4 concentration, and suggested that MD is one of “IgG4-related disease” and distinguishable from SS. Therefore, we clinically and histopathologically examined the disease states of MD and SS in detail.

Materials and methods

Twenty patients with Mikulicz’s disease and 18 with SS were comparatively studied to determine clinical characteristics in MD patients.

Results

Sialography in MD patients did not show the “apple-tree sign” typically seen in SS. Serologically, high serum IgG4 levels but not anti-SS-A or anti-SS-B antibodies were observed in MD. SS showed lymphocytic infiltration of various subsets with atrophy or severe destruction of the acini, while MD showed selective infiltration of IgG4+ plasma cells with hyperplastic germinal centers and mild acini destruction. Corticosteroid treatment of MD reduced IgG and IgG4 levels and improved salivary function. A negative correlation between disease duration and increasing rate of salivary flow was observed in MD.

Conclusions

These results suggested that the pathogenesis of MD might be different from those of SS. Clinical Relevance: early diagnosis and treatment of MD is important for the improvement of salivary function.

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Acknowledgements

This work was supported by “Research on Measures for Intractable Diseases” Project: matching fund subsidy from the Ministry of Health Labour and Welfare, Japan.

Conflict of interest

The authors declare that they have no conflict of interest.

Author information

Authors and Affiliations

  1. Section of Oral and Maxillofacial Oncology, Division of Maxillofacial Diagnostic and Surgical Sciences, Faculty of Dental Science, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan

    Masafumi Moriyama, Akihiko Tanaka, Takashi Maehara, Yukiko Ohyama, Jun-Nosuke Hayashida, Shoichi Shinozaki, Yoshiaki Kubo, Sachiko Furukawa & Seiji Nakamura

  2. Department of Oral and Maxillofacial Radiology, Faculty of Dental Science, Kyushu University, Fukuoka, Japan

    Mayumi Shimizu

  3. Division of Nephrology and Rheumatology, Department of Internal Medicine, Faculty of Medicine, Fukuoka University, Fukuoka, Japan

    Hitoshi Nakashima

  4. Department of Oral and Maxillofacial Surgery, Faculty of Medicine, Fukuoka University, Fukuoka, Japan

    Toshihiro Kikuta

Authors
  1. Masafumi Moriyama
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  2. Akihiko Tanaka
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  3. Takashi Maehara
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  4. Yukiko Ohyama
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  5. Mayumi Shimizu
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  6. Hitoshi Nakashima
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  7. Jun-Nosuke Hayashida
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  8. Shoichi Shinozaki
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  9. Yoshiaki Kubo
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  10. Sachiko Furukawa
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  11. Toshihiro Kikuta
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  12. Seiji Nakamura
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Corresponding author

Correspondence to Seiji Nakamura.

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Moriyama, M., Tanaka, A., Maehara, T. et al. Clinical characteristics of Mikulicz’s disease as an IgG4-related disease. Clin Oral Invest 17, 1995–2002 (2013). https://doi.org/10.1007/s00784-012-0905-z

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  • DOI: https://doi.org/10.1007/s00784-012-0905-z

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