A Rare Case of IgG4-Related Disease Presenting as a Unilateral Severe Dacryoadenitis Complicated by Hypophysitis and Hypertrophic Pachymeningitis.
A Rare Case of IgG4-Related Disease Presenting as a Unilateral Severe Dacryoadenitis Complicated by Hypophysitis and Hypertrophic Pachymeningitis.
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IgG4 相关疾病的罕见病例，表现为单侧严重泪腺炎并发垂体炎和肥厚性硬脑膜炎。
DOI：
10.1097/rhu.0000000000000989
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发表时间：
2020
期刊：
影响因子：
--
通讯作者：
Sugiyama E.
中科院分区：
文献类型：
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作者：
Yoshida Y;Kondo T;Hosokawa Y;Oki K;Yukawa K;Araki K;Kohno H;Kuranobu T;Tokunaga T;Oi K;Sugimoto T;Oda K;Nojima T;Hirata S;Sugiyama E.
入院时生命体征在参考范围内：血压，108/79 mmHg；脉搏，每分钟90次；温度36.8℃；呼吸频率，每分钟16次；氧气饱和度，室内空气97%她有意识，有方向感，对口头命令有反应。她的体重和身高分别为50公斤和155厘米。右眼睑严重肿大，无法完全睁开右眼（图1A）。没有腮腺和下颚腺肿大，结膜苍白和黄疸。肺音清晰，无心脏杂音。腹部平坦柔软，无压痛。神经学检查显示右眼眼外肌运动受限。最初的实验室调查显示嗜酸性粒细胞增多和血清IgG4高水平462.0 mg/dL。补体水平轻度升高。蛋白酶-3、髓过氧化物酶抗核抗体、抗ssa抗体、抗ssb抗体、抗中性粒细胞胞浆抗体均为阴性。结核菌特异性干扰素释放试验结果为阴性。尿液分析显示基本正常。头部和胸部的计算机断层扫描分别显示双侧鼻窦炎和多发肺结节。脑部钆增强磁共振成像（MRI）显示右眼眶假瘤肿胀（图2A），大垂体肿块累及右颞叶柄和肥厚性肿脑膜炎（图2B， C）。右眼眶肿块病理检查显示纤维化和密集淋巴浆浸润，每高倍视场约有100个igg4阳性浆细胞，igg4阳性/ igg阳性比值约为0.9（图3）。根据综合
Her vital signs at the time of admission to the hospital were within reference range: blood pressure, 108/79 mmHg; pulse, 90 beats per minute; temperature, 36.8 C; respiratory rate, 16 breaths per minute; and oxygen saturation, 97% on room air. She was conscious, oriented, and responded to verbal commands. Her weight and height were 50 kg and 155 cm, respectively.Her right eyelid was severely enlarged, and she was unable to open her right eye fully (Fig. 1A). There was no enlargement of the parotid and submandibular glands or conjunctival pallor and jaundice. The lung sounds were clear, and there were no heart murmurs. The abdomen was flat and soft without tenderness. Neurological examination revealed restricted movement of the extraocular muscles of her right eye. The initial laboratory investigations revealed eosinophilia and a high serum IgG4 level of 462.0 mg/dL. Complement level was slightly elevated. Anti-nuclear antibody, anti-SSA antibody, anti-SSB antibody, and anti-neutrophil cytoplasmic antibody of proteinase-3, and myeloperoxidase tests yielded negative results. Tuberculosisspecific interferon gamma release assay yielded a negative result. Urine analysis showed mostly normal values. Computed tomography of the head and chest revealed bilateral rhinosinusitis and multiple lung nodules, respectively. Magnetic resonance imaging (MRI) of the brain with gadolinium enhancement showed swelling of the right orbital pseudotumor (Fig. 2A) and a large pituitary mass involving the stalk and hypertrophic pachymeningitis of the right temporal lobe (Figs. 2B, C). Pathological evaluation of the right orbital mass revealed fibrosis and dense lymphoplasmatic infiltration with approximately 100 IgG4-positive plasma cells per high-power field and an IgG4-positive/IgG-positive ratio of approximately 0.9 (Fig. 3). According to the comprehensive