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Current management of sarcoidosis I: pulmonary, cardiac, and neurologic manifestations

机译:结节病的目前管理I:肺,心脏和神经系统表现

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Purpose of review Sarcoidosis is a systemic disease characterized by noncaseating granulomatous inflammation of multiple organ systems. Pulmonary, cardiac, and neurologic involvements have the worst prognosis. Current recommendations for the therapeutic management and follow-up of sarcoidosis involving these critical organs will be reviewed. Recent findings In those sarcoidosis patients requiring immunosuppressive therapy, corticosteroids are used first at varying doses depending on the presenting manifestation. Patients with symptomatic pulmonary, cardiac, or neurologic involvement will be maintained on corticosteroids for at least a year. Many require a second immunosuppressive agent with methotrexate used most commonly. Anti-tumor necrosis factor agents, especially infliximab, are effective and recommendations for their use have been proposed. Summary Evidence-based treatment guidelines do not exist for most sarcoidosis clinical manifestations. Therefore, clinical care of these patients must rely on expert opinion. Patients are best served by a multidisciplinary approach to their care. Future research to identify environmental triggers, genetic associations, biomarkers for treatment response, and where to position new steroid-sparing immunosuppressive agents is warranted.
机译:审查的目的是一种全身性疾病,其特征在于多器官系统的非肉芽肿性炎症。肺,心脏和神经系统参与具有最严重的预后。将审查涉及这些关键机关的治疗管理和涉及这些关键机关的干扰的后续建议。最近在那些需要免疫抑制治疗的结节病患者中的发现,根据呈现表现,首先以不同剂量使用皮质类固醇。患有症状性肺,心脏或神经系统参与的患者将保持在皮质类固醇上至少一年。许多人需要最常用的甲氨蝶呤的第二种免疫抑制剂。抗肿瘤坏死因子剂,特别是英夫利昔单抗,是有效的,并提出了其使用的建议。大多数结节病的临床表现不存在基于循证的治疗指南。因此,这些患者的临床护理必须依赖专家意见。患者最好通过多学科方法进行护理服务。未来的研究以识别环境触发,遗传关联,治疗反应的生物标志物,并保证新的类固醇味剂免疫抑制剂的位置。

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