Hervas-Garcia J V, Pagani-Cassara F
Hospital Universitari Arnau de Vilanova , Lleida, Espana.
Fundacion Favaloro, Buenos Aires, Argentina.
Rev Neurol. 2019 Jun 16;68(12):524-530. doi: 10.33588/rn.6812.2018261.
Chronic relapsing inflammatory optic neuropathy (CRION) is an inflammatory disease characterized by painful recurrent episodes of optic neuritis with clear response to steroids and relapses with treatment withdrawal.
To performed a systematic review of the literature about CRION, since 2003 to present in medical database. We excluded pediatric or animal research articles. Search terms: CRION, chronic relapsing inflammatory optic neuropathy, recurrent optic neuritis, steroid dependent optic neuritis, and immunosuppression dependent optic neuritis.
CRION is a relapsing optic neuritis of unknown aetiology. The strong response to corticosteroids to avoid recurrence suggests that it might be an immune-mediated disease. CRION typically presents as a subacute and recurrent optic neuropathy with severe visual loss. The principal differential diagnoses are the demyelinating (multiple sclerosis, neuromyelitis optica spectrum disorders and anti-MOG), systemic (mostly sarcoidosis) and infectious diseases. CRION has a dramatic and dependent corticoids response; to avoid adverse events, we use immunosuppressive treatment in long-term.
CRION is a rare, recurrent and cortico-dependent disease of optic nerve. An early diagnosis and accuracy treatment will improve the prognosis.
慢性复发性炎性视神经病变（CRION）是一种炎性疾病，其特征为视神经炎反复发作且伴有疼痛，对类固醇治疗反应明显，停药后复发。
对自2003年至今医学数据库中有关CRION的文献进行系统综述。我们排除了儿科或动物研究文章。检索词：CRION、慢性复发性炎性视神经病变、复发性视神经炎、类固醇依赖性视神经炎和免疫抑制依赖性视神经炎。
CRION是一种病因不明的复发性视神经炎。对皮质类固醇有强烈反应以避免复发，这表明它可能是一种免疫介导的疾病。CRION通常表现为亚急性复发性视神经病变，伴有严重视力丧失。主要鉴别诊断包括脱髓鞘疾病（多发性硬化症、视神经脊髓炎谱系障碍和抗髓鞘少突胶质细胞糖蛋白抗体相关疾病）、全身性疾病（主要是结节病）和感染性疾病。CRION对皮质类固醇有显著且依赖的反应；为避免不良事件，我们长期使用免疫抑制治疗。
CRION是一种罕见的、复发性且依赖皮质类固醇的视神经疾病。早期诊断和准确治疗将改善预后。
