Clinical features of IgG4-related sclerosing dacryoadenitis

1. Satoru Kase
2. Kan Ishijima
3. Mika Noda
4. Susumu Ishida

Hokkaido University Hospital, Hokkaido, Japan

Purpose: IgG4-related disease involves multiple systemic organs, including lacrimal gland. The aim of this study is to examine clinical features of IgG4-related sclerosing dacryoadenitis.
Methods: Seven patients (one male and six females), treated in Hokkaido University Hospital from 2006 to 2009, were retrospectively analyzed based on medical record, regarding ophthalmological findings and clinical course.

Results: The age of onset was 52〜72 years old (mean 61．3). Five were bilateral and two were unilateral cases. Patients complained of upper eyelid swelling in all cases, and blurred vision in one case. Ophthalmic findings were mild corneal epithelial disorder, low lacrimal tear secretion, proptosis, and optic perineuritis. Magnetic resonance imaging revealed swelling of the lacrimal gland. Serum IgG4 levels were high in all cases, measuring 201~1370 mg/dl (mean: 513 mg/dl). Histopathology in the incised lacrimal gland tissues was lympho-plasmacytic infiltration with fibrosis. Immunohistochemistry revealed high IgG4/IgG ratio, over 50%, in infiltrated plasma cells. Systemic complications were bilateral siaoadenitis in three cases, lymphadenopathy in two, and autoimmune pancreatitis in one. High-dose corticosteroid was administrated in one case, and oral corticosteroid of 30 mg was initially performed in four cases, who consequently showed decreased serum IgG4 concentration and reduced lacrimal gland swelling.

Conclusions: IgG4-related sclerosing dacryoadenitis is characterized by high serum IgG4 levels, IgG4-positive plasmacytic infiltration in lacrimal gland tissue, and good response to corticosteroid. Chronic dacryoadenitis may be an initial presentation in IgG4-related disease, because of presence of the dacryoadenitis cases without systemic involvements.