Clinical features of IgG4-related dacryoadenitis

Clinical features of IgG4-related dacryoadenitis
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DOI:
10.1007/s00417-013-2541-y
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发表时间:
2014-03-01
影响因子:
2.7
通讯作者:
Ozaki, Noriko
Ozaki, Noriko
中科院分区:
医学3区
文献类型:
--
作者:
Koizumi, Satomi;Kamisawa, Terumi;Ozaki, Noriko

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为了阐明IgG 4相关性泪腺炎的临床特征,前瞻性研究了12例患者的临床特征、实验室检查、放射学检查、相关疾病、治疗和预后(七男五女;平均年龄60.9 ± 15.1岁),与IgG 4相关的泪腺炎。除了眼睑肿胀,在7名患者中观察到其他眼科症状,包括复视(n = 4)、上睑下垂(n = 2)、视野障碍(n = 2)、眼痛(n = 2)、视力下降(n = 2)、眼球运动障碍(n = 1)、干眼症(n = 1)、角膜溃疡(n = 1)和溢泪(n = 1)。一半患者的泪腺肿胀是双侧的。9例患者中存在其他IgG 4相关疾病,包括涎腺炎(n = 5)、自身免疫性胰腺炎(n = 4)、腹膜后纤维化(n = 2)和淋巴结病(n = 8)。其他IgG 4相关疾病患者的血清IgG 4水平(1070 +/- 813 mg/dl)显著高于无疾病患者(197 +/- 59 mg/dl,p = 0.017)。过敏史和血清IgE水平升高,分别检测到6例患者。8例患者显示炎性延伸超出泪腺,如直肌增厚(n = 6),视神经炎症(n = 2)和球后炎症(n = 3)。7例患者激素治疗有效,但2例患者复发,血清IgG 4水平明显升高,并伴有自身免疫性胰腺炎。IgG 4相关性泪腺炎表现为泪腺以外广泛的炎症,常与其他IgG 4相关性疾病或过敏现象相关,以及激素反应性。
To elucidate the clinical characteristics of IgG4-related dacryoadenitis.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.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.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.