Pediatric Immunology, Hematology and Rheumatology Unit, Necker-Enfants Malades Hospital, Assistance Publique-Hopitaux de Paris, Université de Paris, Paris, France.
Department of Internal Medicine and Clinical Immunology, RHU IMAP, Sorbonne University, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
Rev Med Interne. 2023 May;44(5):227-252. doi: 10.1016/j.revmed.2023.04.002. Epub 2023 May 3.
This French National Diagnostic and Care Protocol (NDPC) includes both pediatric and adult patients with non-infectious chronic uveitis (NICU) or non-infectious recurrent uveitis (NIRU). NICU is defined as uveitis that persists for at least 3 months or with frequent relapses occurring less than 3 months after cessation of treatment. NIRU is repeated episodes of uveitis separated by periods of inactivity of at least 3 months in the absence of treatment. Some of these NICU and NIRU are isolated. Others are associated with diseases that may affect various organs, such as uveitis associated with certain types of juvenile idiopathic arthritis, adult spondyloarthropathies or systemic diseases in children and adults such as Behçet's disease, granulomatoses or multiple sclerosis. The differential diagnoses of pseudo-uveitis, sometimes related to neoplasia, and uveitis of infectious origin are discussed, as well as the different forms of uveitis according to their main anatomical location (anterior, intermediate, posterior or panuveitis). We also describe the symptoms, known physiopathological mechanisms, useful complementary ophthalmological and extra-ophthalmological examinations, therapeutic management, monitoring and useful information on the risks associated with the disease or treatment. Finally, this protocol presents more general information on the care pathway, the professionals involved, patient associations, adaptations in the school or professional environment and other measures that may be implemented to manage the repercussions of these chronic diseases. Because local or systemic corticosteroids are usually necessary, these treatments and the risks associated with their prolonged use are the subject of particular attention and specific recommendations. The same information is provided for systemic immunomodulatory treatments, immunosuppressive drugs, sometimes including anti-TNFα antibodies or other biotherapies. Certain particularly important recommendations for patient management are highlighted in summary tables.
这份法国国家诊断和治疗方案(NDPC)包括患有非传染性慢性葡萄膜炎(NICU)或非传染性复发性葡萄膜炎(NIRU)的儿科和成年患者。NICU 定义为持续至少 3 个月的葡萄膜炎,或在治疗停止后不到 3 个月内频繁复发。NIRU 是指在没有治疗的情况下,葡萄膜炎反复发作,间隔至少 3 个月无活动期。其中一些 NICU 和 NIRU 是孤立的,而其他则与可能影响多种器官的疾病相关,如与某些类型的青少年特发性关节炎、成人脊柱关节病或儿童和成人的系统性疾病(如贝切特病、肉芽肿病或多发性硬化症)相关的葡萄膜炎。本文讨论了假性葡萄膜炎的鉴别诊断,假性葡萄膜炎有时与肿瘤有关,以及感染性葡萄膜炎的不同形式,根据其主要解剖位置(前、中、后或全葡萄膜炎)对葡萄膜炎进行分类。本文还描述了症状、已知的病理生理机制、有用的眼科和眼科以外的补充检查、治疗管理、监测以及与疾病或治疗相关的风险的有用信息。最后,该方案还介绍了更一般的护理路径信息,包括参与的专业人员、患者协会、学校或职业环境的调整以及为管理这些慢性疾病的影响而可能实施的其他措施。由于通常需要局部或全身皮质类固醇治疗,因此这些治疗方法及其长期使用相关的风险是特别关注的重点,并提出了具体的建议。对于系统性免疫调节治疗、免疫抑制药物,有时包括抗 TNFα 抗体或其他生物疗法,也提供了相同的信息。在总结表中突出了一些对患者管理特别重要的建议。