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Clinical features of IgG4-related dacryoadenitis

  • Satomi Koizumi
  • Terumi Kamisawa
  • Sawako Kuruma
  • Taku Tabata
  • Susumu Iwasaki
  • Kazuro Chiba
  • Keigo Setoguchi
  • Shinichiro Horiguchi
  • Noriko Ozaki
Inflammatory Disorders

Abstract

Background

To elucidate the clinical characteristics of IgG4-related dacryoadenitis.

Methods

Clinical features, laboratory findings, radiological findings, associated diseases, treatment, and prognosis were prospectively examined in 12 patients (seven men, five women; mean age, 60.9 ± 15.1 years) with IgG4-related dacryoadenitis.

Results

In addition to eyelid swelling, other ophthalmologic symptoms were observed in seven patients, including diplopia (n = 4), ptosis (n = 2), visual field disturbance (n = 2), eye pain (n = 2), decrease of visual acuity (n = 2), eye-movement disturbance (n = 1), dry eye (n = 1), corneal ulcer (n = 1), and epiphora (n = 1). Swelling of the lacrimal glands was bilateral in half of the patients. Other IgG4-related diseases were present in nine patients, including sialadenitis (n = 5), autoimmune pancreatitis (n = 4), retroperitoneal fibrosis (n = 2), and lymphadenopathy (n = 8). Serum IgG4 levels were significantly higher in patients with other IgG4-related disease (1070 ± 813 mg/dl) than in those without (197 ± 59 mg/dl, p = 0.017). Allergic histories and elevated serum IgE levels were each detected in six patients. Eight patients showed inflammatory extension beyond the lacrimal gland, such as thickened rectus muscle (n = 6), inflammation of the optic nerve (n = 2), and retrobulbar inflammation (n = 3). Steroid therapy was effective in seven patients, but dacryoadenitis relapsed in two patients with markedly higher serum IgG4 levels and autoimmune pancreatitis.

Conclusions

IgG4-related dacryoadenitis showed various ophthalmologic symptoms due to extensive inflammation beyond the lacrimal gland, frequent association with other IgG4-related disease or allergic phenomena, and steroid responsiveness.

Keywords

IgG4 IgG4-related dacryoadenitis Lacrimal gland Mikulicz’s disease Autoimmune pancreatitis 

Notes

Acknowledgments

This work was supported in part by the Research Committee of Intractable Diseases, provided by the Ministry of Health, Labour, and Welfare of Japan.

Conflict of Interest

The authors state that they have no conflict of interest.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Satomi Koizumi
    • 1
  • Terumi Kamisawa
    • 1
  • Sawako Kuruma
    • 1
  • Taku Tabata
    • 2
  • Susumu Iwasaki
    • 1
  • Kazuro Chiba
    • 1
  • Keigo Setoguchi
    • 1
  • Shinichiro Horiguchi
    • 3
  • Noriko Ozaki
    • 4
  1. 1.Department of Internal MedicineTokyo Metropolitan Komagome HospitalBunkyo-kuJapan
  2. 2.Department of EndoscopyTokyo Metropolitan Komagome HospitalBunkyo-kuJapan
  3. 3.Department of PathologyTokyo Metropolitan Komagome HospitalBunkyo-kuJapan
  4. 4.Department of OphthalmologyTokyo Metropolitan Komagome HospitalBunkyo-kuJapan

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