IgG4相关性泪腺和唾液腺炎
IgG4-related dacryoadenitis and sialadenitis
ORPHA:79078疾病亚型
定义 英文原文（暂无中文）
IgG4-related dacryoadenitis and sialoadenitis (Mikulicz disease) is an IgG4-related sclerosing disease characterized by persistent, usually painless, bilateral enlargement of the lacrimal, parotid, and submandibular glands associated with elevated levels of serum immunoglobulin (Ig) G4 and with lymphocyte and IgG4-positive plasmacyte infiltration. It predominantly causes mouth and eye dryness but can also affect other organs such as the lungs, liver, and kidneys, and be accompanied by complications such as autoimmune pancreatitis (AIP), retroperitoneal fibrosis, and tubulointerstitial nephritis.
别名
慢性泪腺和唾液腺炎
基本事实
- 遗传方式
- 不适用
- 发病年龄
- 各年龄段
临床表型 33
极常见 99–80%4
- 唾液腺形态异常 HP:0010286
- 颌下腺异常 HP:0010287
- 泪腺增大 HP:0007734
- 血液IgG4增多 HP:0032300
常见 79–30%1
- 腮腺肿大 HP:0011801
偶见 29–5%19
- 视神经形态异常 HP:0000587
- 眼外肌异常 HP:0008049
- 眼眶异常 HP:0000315
- 抗核抗体阳性 HP:0003493
- 自身免疫 HP:0002960
- 细胞质抗中性粒细胞抗体阳性 HP:0032230
- 面部水肿 HP:0000282
- 疲乏 HP:0012378
- 发热 HP:0001945
- 泛发性肌无力 HP:0003324
- 循环IgA水平升高 HP:0003261
- 循环IgM水平升高 HP:0003496
- 干燥性角结膜炎 HP:0001097
- 肌炎 HP:0100614
- 眼睑水肿 HP:0100540
- 眶周丰满 HP:0000629
- 眼球突出 HP:0000520
- 体重减轻 HP:0001824
- 口腔干燥 HP:0000217
罕见 <4–1%9
- 泪道形态异常 HP:0011481
- 失明 HP:0000618
- 淋巴结肿大 HP:0002716
- 结节性甲状腺肿 HP:0005994
- 视神经受压 HP:0007807
- 腹膜后纤维化 HP:0005200
- 血小板减少症 HP:0001873
- 甲状腺炎 HP:0100646
- 肾小管间质性肾炎 HP:0001970
外部标识与链接
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本页数据来源
- 疾病定义、同义词、基因、表型、流行病学：Orphanet（CC BY 4.0）
- 中文病名：Orphanet 中文包，冻结于 2020-06-01
- 表型中文标签：HPO 简体中文翻译（CHPO 上游成果）