- Email: [email protected]
Mikulicz Disease Mimicking Intraorbital Tumors
Accepted Manuscript Mikulicz’s disease mimicking intraorbital tumors Satoshi Murai, MD, Mitsuo Kuriyama, MD, Kaoru Terasaka, MD PII:
S1878-8750(17)31689-3
DOI:
10.1016/j.wneu.2017.09.173
Reference:
WNEU 6617
To appear in:
World Neurosurgery
Received Date: 4 May 2017 Revised Date:
23 September 2017
Accepted Date: 25 September 2017
Please cite this article as: Murai S, Kuriyama M, Terasaka K, Mikulicz’s disease mimicking intraorbital tumors, World Neurosurgery (2017), doi: 10.1016/j.wneu.2017.09.173. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Title Mikulicz’s disease mimicking intraorbital tumors
1) Department of Neurosurgery, Kure Kyosai Hospital, Kure, Japan
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Corresponding author and address
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Satoshi Murai, MD1, Mitsuo Kuriyama, MD1, and Kaoru Terasaka, MD1
Satoshi Murai, MD
Department of Neurological Surgery, Okayama University Graduate School of Medicine, 700-8558, Japan TEL: +81-86-235-7336 FAX: +81-86-227-0191 E-mail: [email protected]
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Abstract
M AN U
Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama City, Okayama
We report a case of Mikulicz’s disease (MD), an immunoglobulin G4 (IgG4)-related disease that affects the lacrimal and salivary glands. IgG4-related disease is a relatively new clinical entity, and not commonly encountered by neurosurgeons. MD sometimes mimics intraorbital tumors such as malignant lymphoma, but responds well to corticosteroid treatment. Thus, it is important
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to recognize the clinical and radiographic features of MD.
A 68-year-old woman presented with bilateral swelling of her upper eyelids and difficulty opening her eyes. Physical examination showed bilateral palpebral swelling, but impairment of ocular movement and double vision were not described. Magnetic resonance imaging (MRI) revealed marked symmetric enlargement of the bilateral lacrimal glands with hypointensities on T1-weighted images (Fig. A, B), and isointensities on T2-weighted fat-saturated images (Fig. C, D). Laboratory data showed an elevated serum immunoglobulin G4 (IgG4) level of 595 mg/dl (normal range, 4–108 mg/dl), and no elevation of tumor markers. Thus, a diagnosis of Mikulicz’s disease (MD) was made. MD is an IgG4-related disease that affects the lacrimal and salivary glands, and responds well to corticosteroid treatment. After 8 weeks of treatment with prednisolone, serum IgG4 levels were decreased, and MRI showed marked improvement of the
ACCEPTED MANUSCRIPT
swollen lacrimal glands. Keywords
References 1.
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Bilateral swelling of lacrimal glands; IgG4-related disease; Mikulicz’s disease
Kamisawa T, Funata N, Hayashi Y, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38:982-4.
Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366:539-51.
3.
Umehara H, Okazaki K, Masaki Y, et al. A novel clinical entity, IgG4-related disease
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2.
(IgG4RD): general concept and details. Mod Rheumatol. 2012;22:1-14.
Yamamoto M, Takahashi H, Ohara M, et al. A new conceptualization for Mikulicz’s disease
M AN U
4.
as an IgG4-related plasmacytic disease. Mod Rheumatol. 2006;16:335-40. Goto H, Takahira M, Azumi A, et al. Diagnostic criteria for IgG4-related ophthalmic
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disease. Jpn J Ophthalmol. 2015;59:1-7.
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Abbreviations:
MD (Mikulicz’s disease) IgG4 (immunoglobulin G4)
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MRI (magnetic resonance imaging)
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Conflicts of interest: none
S1878-8750(17)31689-3
DOI:
10.1016/j.wneu.2017.09.173
Reference:
WNEU 6617
To appear in:
World Neurosurgery
Received Date: 4 May 2017 Revised Date:
23 September 2017
Accepted Date: 25 September 2017
Please cite this article as: Murai S, Kuriyama M, Terasaka K, Mikulicz’s disease mimicking intraorbital tumors, World Neurosurgery (2017), doi: 10.1016/j.wneu.2017.09.173. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Title Mikulicz’s disease mimicking intraorbital tumors
1) Department of Neurosurgery, Kure Kyosai Hospital, Kure, Japan
SC
Corresponding author and address
RI PT
Satoshi Murai, MD1, Mitsuo Kuriyama, MD1, and Kaoru Terasaka, MD1
Satoshi Murai, MD
Department of Neurological Surgery, Okayama University Graduate School of Medicine, 700-8558, Japan TEL: +81-86-235-7336 FAX: +81-86-227-0191 E-mail: [email protected]
TE D
Abstract
M AN U
Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama City, Okayama
We report a case of Mikulicz’s disease (MD), an immunoglobulin G4 (IgG4)-related disease that affects the lacrimal and salivary glands. IgG4-related disease is a relatively new clinical entity, and not commonly encountered by neurosurgeons. MD sometimes mimics intraorbital tumors such as malignant lymphoma, but responds well to corticosteroid treatment. Thus, it is important
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Manuscript
EP
to recognize the clinical and radiographic features of MD.
A 68-year-old woman presented with bilateral swelling of her upper eyelids and difficulty opening her eyes. Physical examination showed bilateral palpebral swelling, but impairment of ocular movement and double vision were not described. Magnetic resonance imaging (MRI) revealed marked symmetric enlargement of the bilateral lacrimal glands with hypointensities on T1-weighted images (Fig. A, B), and isointensities on T2-weighted fat-saturated images (Fig. C, D). Laboratory data showed an elevated serum immunoglobulin G4 (IgG4) level of 595 mg/dl (normal range, 4–108 mg/dl), and no elevation of tumor markers. Thus, a diagnosis of Mikulicz’s disease (MD) was made. MD is an IgG4-related disease that affects the lacrimal and salivary glands, and responds well to corticosteroid treatment. After 8 weeks of treatment with prednisolone, serum IgG4 levels were decreased, and MRI showed marked improvement of the
ACCEPTED MANUSCRIPT
swollen lacrimal glands. Keywords
References 1.
RI PT
Bilateral swelling of lacrimal glands; IgG4-related disease; Mikulicz’s disease
Kamisawa T, Funata N, Hayashi Y, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38:982-4.
Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366:539-51.
3.
Umehara H, Okazaki K, Masaki Y, et al. A novel clinical entity, IgG4-related disease
SC
2.
(IgG4RD): general concept and details. Mod Rheumatol. 2012;22:1-14.
Yamamoto M, Takahashi H, Ohara M, et al. A new conceptualization for Mikulicz’s disease
M AN U
4.
as an IgG4-related plasmacytic disease. Mod Rheumatol. 2006;16:335-40. Goto H, Takahira M, Azumi A, et al. Diagnostic criteria for IgG4-related ophthalmic
EP
TE D
disease. Jpn J Ophthalmol. 2015;59:1-7.
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Abbreviations:
MD (Mikulicz’s disease) IgG4 (immunoglobulin G4)
AC C
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TE D
M AN U
SC
RI PT
MRI (magnetic resonance imaging)
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Conflicts of interest: none