Neri Piergiorgio, Ricci Federico, Giovannini Alfonso, Arapi Ilir, De Felici Cecilia, Cusumano Andrea, Mariotti Cesare
Ocular Immunology Service, The Eye Clinic, Polytechnic University of Marche, Azienda Ospedaliera Universitaria-Ospedali Riuniti di Ancona, Via Conca 71, 60020, Ancona, Torrette, Italy,
Int Ophthalmol. 2014 Apr;34(2):359-64. doi: 10.1007/s10792-013-9801-z. Epub 2013 Jun 14.
To report a case of overlapping choriocapillaritis that initially presented as multifocal choroiditis (MFC) but later showed features compatible with acute zonal occult outer retinopathy (AZOOR) resistant to standard immunosuppression that responded only to adalimumad therapy. A 41-year-old patient presented with multiple small, discrete yellow-whitish spots in both eyes, compatible with MFC. A few weeks later, despite treatment with sub-Tenon and systemic corticosteroids, a choroidal neovascularization occurred in the right eye. The patient was treated with intravitreal anti-vascular endothelial growth factor. After 2 months, reduced visual acuity, photopsia and visual field defect in the left eye occurred. Spectral domain optical coherence tomography revealed photoreceptor outer segment defects common to all choriocapillaritis. The additional finding of an annular scotoma and a 360° ring on indocyanine green angiography led us to make the diagnosis of presumed AZOOR. Despite the combination of several immunosuppressive agents leading to temporary control of the disease, the patient experienced a further worsening. At that point, adalimumab was introduced, which led to an obvious improvement. This case supports the hypothesis that two different entities of the so-called AZOOR complex can be possible in the same eye, even asynchronously. In our case, anti-tumor necrosis factor alpha monoclonal antibody therapy represented a valid treatment option in a patient unresponsive to traditional immunosuppressive treatments.
报告一例重叠性脉络膜毛细血管炎病例,该病例最初表现为多灶性脉络膜炎(MFC),但后来显示出与急性区域性隐匿性外层视网膜病变(AZOOR)相符的特征,对标准免疫抑制治疗耐药,仅对阿达木单抗治疗有反应。一名41岁患者双眼出现多个小的、离散的黄白色斑点,符合MFC表现。几周后,尽管接受了球周和全身皮质类固醇治疗,右眼仍发生了脉络膜新生血管。患者接受了玻璃体内抗血管内皮生长因子治疗。2个月后,左眼出现视力下降、闪光感和视野缺损。光谱域光学相干断层扫描显示所有脉络膜毛细血管炎均存在的光感受器外段缺陷。吲哚菁绿血管造影检查发现的额外环形暗点和360°环使我们做出了疑似AZOOR的诊断。尽管联合使用了多种免疫抑制剂使病情得到了暂时控制,但患者病情仍进一步恶化。此时引入了阿达木单抗,病情明显改善。该病例支持这样一种假设,即所谓的AZOOR综合征的两种不同实体可能在同一只眼中出现,甚至不同步出现。在我们的病例中,抗肿瘤坏死因子α单克隆抗体治疗是对传统免疫抑制治疗无反应患者的一种有效治疗选择。