Relapsing polychondritis: A 2016 update on clinical features, diagnostic tools, treatment and biological drug use
Relapsing polychondritis: A 2016 update on clinical features, diagnostic tools, treatment and biological drug use
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DOI：
10.1016/j.berh.2016.08.001
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发表时间：
2016-04-01
影响因子：
5.2
通讯作者：
Amoura, Zahir
中科院分区：
文献类型：
--
作者：
Mathian, Alexis;Miyara, Makoto;Amoura, Zahir
复发性多软骨炎(RP)是一种非常罕见的自身免疫性疾病，其特征是软骨组织(关节、耳朵、鼻子、间盘、喉、气管和软骨支气管)的复发性炎症，可进展为长期的软骨萎缩和/或畸形。非软骨组织也可能受到影响，如眼睛、心脏、主动脉、内耳和皮肤。RP有一个漫长且不可预测的过程。由于没有随机的治疗试验，RP的治疗仍然主要是经验性的。轻微的疾病可以用非类固醇抗炎药治疗，而更严重的疾病可以用全身皮质类固醇治疗。危及生命的疾病和皮质类固醇依赖或耐药疾病是免疫抑制治疗的适应症，如甲氨蝶呤、硫唑嘌呤、霉酚酸酯和环磷酰胺。尽管使用类固醇和免疫抑制药物，生物制品仍可作为活动期疾病患者的二线治疗。虽然生物制剂代表了治疗RP的新的潜在治疗方法，但关于它们在RP中的应用，非常稀有的信息可用来得出任何确定的结论。(C)2016爱思唯尔有限公司。保留所有权利。
Relapsing polychondritis (RP) is a very rare autoimmune disease characterised by a relapsing inflammation of the cartilaginous tissues (joints, ears, nose, intervertebral discs, larynx, trachea and cartilaginous bronchi), which may progress to long-lasting atrophy andior deformity of the cartilages. Non-cartilaginous tissues may also be affected, such as the eyes, heart, aorta, inner ear and skin. RP has a long and unpredictable course. Because no randomised therapeutic trials are available, the treatment of RP remains mainly empirical. Minor forms of the disease can be treated with non steroidal anti-inflammatory drugs, whereas more severe forms are treated with systemic corticosteroids. Life-threatening diseases and corticosteroid-dependent or resistant diseases are an indication for immunosuppressant therapy such as methotrexate, azathioprine, mycophenolate mofetil and cyclophosphamide. Biologics could be given as second-line treatment in patients with an active disease despite the use of steroids and immunosuppressive drugs. Although the biologics represent new potential treatment for RP, very scarce information is available to draw any firm conclusion on their use in RP. (C) 2016 Elsevier Ltd. All rights reserved.