Matsuo Toshihiko, Tanaka Takehiro, Tsuji Kenji
Ophthalmology, Graduate School of Interdisciplinary Science and Engineering in Health Systems, Okayama University, Okayama, JPN.
Ophthalmology, Okayama University Hospital, Okayama, JPN.
Cureus. 2024 Oct 4;16(10):e70865. doi: 10.7759/cureus.70865. eCollection 2024 Oct.
Immunoglobulin G4 (IgG4)-related disease is characterized by infiltration with IgG4-producing plasma cells in different organs and the elevation of serum IgG4. We present a patient with polycystic kidney disease in long-term follow-up who developed bilateral lacrimal gland enlargement and presumed IgG4-related choroidopathy at different time points. A 45-year-old woman developed bilateral upper eyelid swelling. Head MRI showed bilateral lacrimal gland enlargement, and the resection on both sides revealed foci of infiltration with lymphocytes and plasma cells in bilateral lacrimal glands. The IgG4-immunostaining did not satisfy the diagnostic criteria. She had been taking oral valsartan 40 mg daily for hypertension with polycystic kidney disease. The patient was well until the age of 49 years, when she noticed yellowish vision in the right eye compared to the left eye. The right eye showed multiple yellowish spotty lesions in the deep retina to choroid with a mildly hyperemic optic disc, while the left eye showed the normal fundus. No inflammation was noted in the anterior segments of both eyes. Fundus angiography demonstrated early-phase no-filling with late-phase leakage by fluorescein dye and both early-phase and late-phase no-filling by indocyanine green dye, leading to the diagnosis of acute posterior multifocal placoid pigment epitheliopathy (APMPPE). She began to have oral prednisolone tapered from 30 mg daily and discontinued in a year. At the age of 52 years, she switched to candesartan 8 mg daily and began to have tolvaptan (a selective competitive vasopressin receptor 2 (V) antagonist) 90 mg daily for polycystic kidney disease with liver cysts. At that time, the lesions in the right eye had mild degeneration. The patient was followed once a year ophthalmologically to maintain good vision. At 57 years, serum IgG4, which was measured for the first time on suspicion of IgG4-related disease, was elevated to 269.6 mg/dL. In the following four years to the latest visit at 61 years, she kept stable but high levels of serum IgG4 around 300 mg/dL. Serum IgG4 measurement is helpful to make a clinical diagnosis and, hence, a clinical decision since the spectrum of IgG4-related disease remains obscure.
免疫球蛋白G4（IgG4）相关疾病的特征是不同器官中产生IgG4的浆细胞浸润以及血清IgG4升高。我们报告了一名长期随访的多囊肾病患者，其在不同时间点出现双侧泪腺肿大，并推测患有IgG4相关脉络膜病变。一名45岁女性出现双侧上眼睑肿胀。头部MRI显示双侧泪腺肿大，双侧切除显示双侧泪腺有淋巴细胞和浆细胞浸润灶。IgG4免疫染色不符合诊断标准。她因多囊肾病合并高血压每天口服40毫克缬沙坦。该患者一直情况良好，直到49岁时，她注意到右眼与左眼相比有视物发黄的症状。右眼在视网膜深层至脉络膜可见多个黄色斑点状病变，视盘轻度充血，而左眼眼底正常。双眼前段均未发现炎症。眼底血管造影显示荧光素染料早期充盈缺损、晚期渗漏，吲哚菁绿染料早期和晚期均无充盈，从而诊断为急性后极部多灶性鳞状色素上皮病变（APMPPE）。她开始口服泼尼松龙，剂量从每日30毫克逐渐减量，一年内停药。52岁时，她改用每日8毫克坎地沙坦，并开始因多囊肾病合并肝囊肿每日服用90毫克托伐普坦（一种选择性竞争性血管加压素2型受体拮抗剂）。当时，右眼病变有轻度退变。该患者每年接受一次眼科随访以保持良好视力。57岁时，因怀疑IgG4相关疾病首次检测血清IgG4，其升高至269.6毫克/分升。在接下来直到61岁最新一次就诊的四年里，她的血清IgG4水平一直稳定但较高，约为300毫克/分升。由于IgG4相关疾病的谱仍不明确，血清IgG4检测有助于做出临床诊断，从而做出临床决策。
