Turno-Kręcicka A, Tomczyk-Socha M, Zimny A
Department of Ophthalmology, Wrocław Medical University, Wroclaw, Poland.
Department of Ophthalmology, Wrocław Medical University, Wroclaw, Poland
Lupus. 2016 Dec;25(14):1610-1614. doi: 10.1177/0961203316646464. Epub 2016 May 13.
Progressive outer retinal necrosis syndrome (PORN) is a severe clinical variant of necrotizing herpetic chorioretinitis, which occurs almost exclusively in patients with advanced acquired immunodeficiency syndrome (AIDS). To date, only a few cases of PORN have been reported in patients, mostly among those who were immunocompromised. To our knowledge, only one case of PORN in a patient with systemic lupus erythematosus (SLE) has been described. We report the case of a 44-year old HIV-negative patient with lupus nephritis, whom was being treated by mycophenolate mophetil (MMF), arechin and prednisone. After 14 months of MMF therapy, the patient revealed PORN symptoms; and several months later, the patient developed Type B primary central nervous system lymphoma (PCNSL). PORN is usually compared to acute retinal necrosis (ARN) syndrome, because of having the same causative agent: varicella zoster virus (VZV). There are also some similarities in clinical findings. Our observation supports the hypothesis that PORN symptoms in HIV-negative patients can be an intermediate form between ARN and PORN, and can vary according to the patient's immune status.
进行性外层视网膜坏死综合征（PORN）是坏死性疱疹性脉络膜视网膜炎的一种严重临床变体，几乎仅发生于晚期获得性免疫缺陷综合征（AIDS）患者。迄今为止，仅报道了少数PORN患者病例，大多为免疫功能低下者。据我们所知，仅描述过1例系统性红斑狼疮（SLE）患者发生PORN。我们报告1例44岁狼疮性肾炎的HIV阴性患者，正在接受霉酚酸酯（MMF）、阿昔洛韦和泼尼松治疗。MMF治疗14个月后，该患者出现PORN症状；数月后，该患者发生B型原发性中枢神经系统淋巴瘤（PCNSL）。由于病因相同——水痘带状疱疹病毒（VZV），PORN通常与急性视网膜坏死（ARN）综合征相比较。临床发现也存在一些相似之处。我们的观察支持以下假说：HIV阴性患者的PORN症状可能是ARN和PORN之间的一种中间形式，并可根据患者的免疫状态而有所不同。
