文摘
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?±-5.1?years) with IgG4-related dacryoadenitis. Results In addition to eyelid swelling, other ophthalmologic symptoms were observed in seven patients, including diplopia (n--), ptosis (n--), visual field disturbance (n--), eye pain (n--), decrease of visual acuity (n--), eye-movement disturbance (n--), dry eye (n--), corneal ulcer (n--), and epiphora (n--). Swelling of the lacrimal glands was bilateral in half of the patients. Other IgG4-related diseases were present in nine patients, including sialadenitis (n--), autoimmune pancreatitis (n--), retroperitoneal fibrosis (n--), and lymphadenopathy (n--). Serum IgG4 levels were significantly higher in patients with other IgG4-related disease (1070?±-13?mg/dl) than in those without (197?±-9?mg/dl, p--.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--), inflammation of the optic nerve (n--), and retrobulbar inflammation (n--). 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.