Granulomatous uveitis secondary to IgG4-related disease.
Granulomatous uveitis secondary to IgG4-related disease.
复制标题
继发于IgG4相关疾病的肉芽肿性葡萄膜炎。
DOI：
10.1093/rap/rkab084
复制
发表时间：
2021
影响因子：
3.1
通讯作者：
Stone JH
中科院分区：
文献类型：
--
作者：
Katz G;Harvey L;Stone JH
尊敬的编辑，一位69岁的男性IgG 4相关疾病（IgG 4-RD）患者因左眼疼痛、发红和流泪到急诊科就诊。在此之前6年，患者出现厌食症、12.5 kg的意外体重减轻和口干。他当时被诊断为新发2型糖尿病。胸部CT扫描显示肺部多灶性网状结节性混浊。胸部、腹部和盆腔的PET-CT显示颈部、腋窝、纵隔、肺门、盆腔和腹股沟淋巴结肿大，为氟脱氧葡萄糖avid。在肾下腹主动脉和胰头中也存在氟脱氧葡萄糖亲合力。在评估期间，患者出现进行性肾功能不全和轻度蛋白尿。他的血清肌酐峰值为3.3 mg/dl。腹部CT扫描显示肾皮质呈条纹状低密度外观。血清IgG 4浓度为767 mg/dl（参考：4-86 mg/dl），补体成分C3和C4的水平严重降低：C3 50 mg/dl（81-157 mg/dl）和C4< 6 mg/dl（12-39 mg/dl）。肾活检显示重度慢性活动性间质性肾炎，每个高倍视野> 10个IgG 4+浆细胞。他符合2019年ACR/EULAR IgG 4-RD分类标准[1]，并被诊断为患有该疾病。他开始泼尼松减量，在2个月内淋巴结病和肺部阴影几乎完全消退。他的肾功能和血清补体水平也恢复正常。
Dear Editor, A 69-year-old male with IgG4-related disease (IgG4-RD) presented to the emergency department with left eye pain, redness and tearing. Six years before this presentation, the patient developed anorexia, a 12.5 kg unintentional weight loss and dry mouth. He was diagnosed at that time as having new-onset type 2 diabetes mellitus. A CT scan of the chest demonstrated multifocal reticulonodular opacities in the lungs. PET-CT of the chest, abdomen and pelvis demonstrated cervical, axillary, mediastinal, hilar, pelvic and inguinal adenopathy that was fluorodeoxyglucose avid. Fluorodeoxyglucose avidity was also present in the infrarenal abdominal aorta and in the head of the pancreas.During that evaluation, the patient developed progressive renal insufficiency and mild proteinuria. His serum creatinine peaked at 3.3 mg/dl. A CT scan of the abdomen demonstrated a striated, hypodense appearance of the renal cortices. The serum IgG4 concentration was 767 mg/dl (reference: 4–86 mg/dl), and the levels of complement components C3 and C4 were profoundly depressed: C3 50 mg/dl (81–157 mg/dl) and C4< 6 mg/dl (12–39 mg/dl). A kidney biopsy demonstrated severe chronic active interstitial nephritis with> 10 IgG4+ plasma cells per high power field. He met the 2019 ACR/EULAR classification criteria for IgG4-RD [1] and was diagnosed as having that condition. He began a prednisone taper and experienced the nearly complete resolution of his lymphadenopathy and pulmonary opacities within 2 months. His renal function and serum complement levels also normalized.
登录
查看更多内容
影响因子：
3.1
作者：
Agrawal RV;Murthy S;Sangwan V;Biswas J
通讯作者：
Biswas J
影响因子：
1.6
作者：
Knecht, Pascal B.;Papadia, Marina;Herbort, Carl P.
通讯作者：
Herbort, Carl P.
影响因子：
0.6
作者：
Herbort CP
通讯作者：
Herbort CP
影响因子：
1.7
作者：
Harthan JS;Opitz DL;Fromstein SR;Morettin CE
通讯作者：
Morettin CE
影响因子：
27.4
作者：
Wallace, Zachary S.;Naden, Ray P.;Zhang, Wen
通讯作者：
Zhang, Wen