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Clinical features and symptoms of IgG4-related ophthalmic disease: a multicenter study

  • Clinical Investigation
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Abstract

Purpose

The aim of this study was to elucidate the clinical features and symptoms of IgG4-related ophthalmic disease (IgG4-ROD).

Study design

Retrospective, multicenter study.

Methods

The medical charts of 378 patients with IgG4-ROD diagnosed at 9 hospitals in Japan were reviewed. The demographic profiles, clinical findings, and ocular symptoms of the patients were analyzed.

Results

On the basis of the diagnostic criteria for IgG4-ROD, the diagnosis was definite in 261 patients (69%), probable in 45 patients (12%), and possible in 72 patients (19%). The patients’ mean age at the time of diagnosis was 60.6 ± 13.9 years; 195 (52%) were male. The mean IgG4 serum level at the time of the initial diagnosis was 578.9 mg/dL. Imaging studies showed pathologic lesions as follows: lesions in the lacrimal glands (86%), extraocular muscles (21%), trigeminal nerve (20%), and eyelids (12%); isolated orbital mass (11%); diffuse orbital lesion (8%); lesion in the perioptic nerve (8%); and lesion in the sclera (1%). The ophthalmic symptoms included dry eye (22%), diplopia (20%), decreased vision (8%), and visual field defects (5%). IgG4-ROD with extraocular lesions was observed in 182 patients (48%).

Conclusion

Although the lacrimal glands are well known to be the major pathologic site of IgG4-ROD, various ocular tissues can be affected and cause ophthalmic symptoms including visual loss.

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Acknowledgements

This work was supported by the Japanese Ministry of Health, Labor and Welfare Research Program on Rare and Intractable Diseases, grant number JPMH20FC1040.

Author information

Author notes

  1. Hiroshi Goto and Shun-ichiro Ueda contributed equally to this work.

Authors and Affiliations

  1. Department of Ophthalmology, Tokyo Medical University, 6-7-1 Nishi-shinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan

    Hiroshi Goto, Shun-ichiro Ueda & Rei Nemoto

  2. Okayama Medical Center, Okayama, Japan

    Koh-ichi Ohshima

  3. Mitoyo General Hospital, Kagawa, Japan

    Yuka Sogabe

  4. Kanazawa Medical University, Kanazawa, Japan

    Kazuko Kitagawa

  5. Keio University, Tokyo, Japan

    Yoko Ogawa

  6. Niigata University, Niigata, Japan

    Tokuhide Oyama

  7. Fukushima Medical University, Fukushima, Japan

    Minoru Furuta

  8. Kobe Kaisei Hospital, Kobe, Japan

    Atsushi Azumi

  9. Kanazawa University, Kanazawa, Japan

    Masayuki Takahira

Authors
  1. Hiroshi Goto
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  2. Shun-ichiro Ueda
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  3. Rei Nemoto
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  4. Koh-ichi Ohshima
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  5. Yuka Sogabe
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  6. Kazuko Kitagawa
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  7. Yoko Ogawa
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  8. Tokuhide Oyama
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  9. Minoru Furuta
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  10. Atsushi Azumi
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  11. Masayuki Takahira
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Corresponding author

Correspondence to Hiroshi Goto.

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Conflict of interest

H. Goto, None; S. Ueda, None; R. Nemoto, None; K. Ohshima, None; Y. Sogabe, None; K. Kitagawa, None; Y. Ogawa, None; T. Oyama, None; M. Furuta, None; A. Azumi, None; M. Takahira, None.

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Corresponding Author: Hiroshi Goto

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Goto, H., Ueda, Si., Nemoto, R. et al. Clinical features and symptoms of IgG4-related ophthalmic disease: a multicenter study. Jpn J Ophthalmol 65, 651–656 (2021). https://doi.org/10.1007/s10384-021-00847-3

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  • DOI: https://doi.org/10.1007/s10384-021-00847-3

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