| Three Cases of Hyper-IgG4 Syndrome Involving Ocular Adnexa. |
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KoEun Kim, Min Joung Lee, Nam Ju Kim, Ho Kyung Choung, Sang In Khwarg |
1Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea. resourceful@hanmail.net
2Department of Ophthalmology, Bundang Hospital, Seoul National University, Seongnam, Korea.
3Department of Ophthalmology, Boramae Hospital, Seoul National University, Seoul, Korea. |
| 눈부속기를 침범한 Hyper-IgG4 증후군 3예 |
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김고은1ㆍ이민정1ㆍ김남주1,2ㆍ정호경1,3ㆍ곽상인1 |
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Department of Ophthalmology, Seoul National University College of Medicine1, Seoul, Korea Department of Ophthalmology, Bundang Hospital, Seoul National University2, Seongnam, Korea Department of Ophthalmology, Boramae Hospital, Seoul National University3, Seoul, Korea |
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| Abstract |
PURPOSE
To report 3 cases of hyper-IgG4 syndrome involving ocular adnexa. CASE SUMMARY: A 66-year-old woman presented with swelling of the bilateral upper eyelids with ocular pain that began 1 year before. Nodular episcleral injection of the left eye and other generalized symptoms, such as cough, decreased hearing ability, multiple nodular lesions of the bilateral lungs and right kidney, together suggested rheumatic disease. Orbital computed tomographic images revealed diffuse swelling of the bilateral lacrimal glands. After immunostaining a surgically-biopsied specimen from the lacrimal gland for IgG4 expression, 15% of infiltrated lymphoplasmacytic cells were IgG4-positive. Similar findings were shown with biopsied specimens from the lung and kidney; therefore, the patient was diagnosed with Hyper-IgG4 syndrome. A 49-year-old woman complained of a mass in the left upper eyelid that began 4 years earlier. Orbital computed tomographic images showed a 5-mm-sized mass in the left upper eyelid. Ocular adnexal Hyper-IgG4 syndrome was confirmed by the immunostained biopsy from the left upper eyelid, showing infiltration of IgG4-positive lymphoplasmacytic cells. A 51-year-old woman presented with swelling of the bilateral lacrimal glands. Enlargement of the bilateral lacrimal glands were apparent in orbital computed tomographic images. After anti-IgG4 antibody staining of a biopsied specimen from the right lacrimal gland, dense infiltration of IgG4-positive lymphoplasmacytic cells was observed. The patient was also diagnosed with Hyper-IgG4 syndrome. |
| Key Words:
Autoimmune pancreatitis;Hyper-IgG4 syndrome;IgG4-positive lymphoplasmacytic cells;Lacrimal gland;Ocular adnexa |
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