Retina Consultants of Austin and Retina Research Center, 3705 Medical Parkway, Suite 410, Austin, TX, 78705, USA.
Department of Ophthalmology, The University of Texas at Austin Dell Medical School, Austin, TX, USA.
J Med Case Rep. 2020 Jul 5;14(1):89. doi: 10.1186/s13256-020-02431-8.
Immunoglobulin G4-related disease is a recently recognized condition with pathologic features that are consistent across a wide range of organ systems. Orbital manifestations of this disease entity typically involve the lacrimal gland and lacrimal duct, extraocular muscles, orbital soft tissue, and sclera. Here, the authors report the first known case of inflammatory choroidal neovascular membrane associated with immunoglobulin G4-related disease and offer suggestions for clinical management of this enigmatic condition.
A 38-year-old Caucasian man with a history of recurrent tonsillitis presented with blurry vision in his left eye of 1-week duration and was diagnosed as having inflammatory choroidal neovascular membrane. An infectious workup was negative, but his serum immunoglobulin G4 level was elevated at 248 mg/dL (reference 4-86), and a subsequent tonsillectomy for a repeat episode of tonsillitis revealed increased immunoglobulin G4 staining on histopathology, thus confirming the diagnosis of immunoglobulin G4-related disease. The inflammatory choroidal neovascular membrane was treated with intravitreal bevacizumab injections and orally administered prednisone resulting in improved visual acuity and choroidal neovascular membrane regression. He later received rituximab infusions for immunoglobulin G4-related disease.
We report a case of choroidal neovascularization associated with immunoglobulin G4-related disease, a chronic inflammatory condition whose ophthalmic manifestations typically include dacryoadenitis, orbital myositis, or scleritis. This is the first reported instance of inflammatory choroidal neovascular membrane associated with immunoglobulin G4-related disease. Early detection of this disease is important to avoid organ damage and potential complications, so clinicians should maintain an index of suspicion for this condition when inflammatory choroidal neovascular membrane is observed.
免疫球蛋白 G4 相关疾病是一种新认识的疾病，其病理特征在广泛的器官系统中具有一致性。这种疾病实体的眼眶表现通常涉及泪腺和泪管、眼外肌、眼眶软组织和巩膜。在这里，作者报告了首例与免疫球蛋白 G4 相关疾病相关的炎症性脉络膜新生血管膜，并为这种神秘疾病的临床管理提供了建议。
一名 38 岁的白种男性，有反复发作的扁桃体炎病史，左眼视力模糊 1 周，被诊断为炎症性脉络膜新生血管膜。感染性检查结果为阴性，但他的血清免疫球蛋白 G4 水平升高至 248mg/dL（参考值 4-86），随后因反复发作的扁桃体炎行扁桃体切除术，发现组织病理学上免疫球蛋白 G4 染色增加，从而确诊为免疫球蛋白 G4 相关疾病。炎症性脉络膜新生血管膜采用玻璃体内注射贝伐单抗和口服泼尼松龙治疗，视力和脉络膜新生血管膜均得到改善。他后来接受了利妥昔单抗治疗免疫球蛋白 G4 相关疾病。
我们报告了一例与免疫球蛋白 G4 相关疾病相关的脉络膜新生血管化病例，这是一种慢性炎症性疾病，其眼部表现通常包括泪腺炎、眼眶肌炎或巩膜炎。这是首例报告的与免疫球蛋白 G4 相关疾病相关的炎症性脉络膜新生血管膜。早期发现这种疾病对于避免器官损伤和潜在并发症非常重要，因此当观察到炎症性脉络膜新生血管膜时，临床医生应保持对这种疾病的怀疑指数。
