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[结节病的治疗]

[Treatment of sarcoidosis].

作者信息

Londner C, Zendah I, Freynet O, Carton Z, Dion G, Nunes H, Valeyre D

机构信息

EA 2363, service de pneumologie, hôpital Avicenne, Assistance publique-Hôpitaux de Paris, université Paris 13, 125, rue de Stalingrad, 93009 Bobigny, France.

出版信息

Rev Med Interne. 2011 Feb;32(2):109-13. doi: 10.1016/j.revmed.2010.10.351. Epub 2010 Dec 14.

Abstract

Sarcoidosis is a common disorder with heterogeneous severity. Corticosteroids are the cornerstone of its treatment and allow a disease remission but only with a suspensive effect. Immunosuppressive drugs, hydroxychloroquine and infliximab may be useful in some patients. Half of the patients recover spontaneously without any treatment. In the remaining patients, a treatment is necessary either at presentation or during the follow-up in the presence of a disease flare. Treatment duration should be of at least 12 months. The main indications of a systemic treatment include ophthalmologic, neurologic, cardiovascular, renal, laryngeal involvements, severe pulmonary manifestations, lupus pernio and marked hypercalcaemia. Initially, patients should receive prednisone or prednisolone at 0.5 to 1 mg/kg daily for 6 to 12 weeks to obtain a complete remission, and then followed by a gradual dose reduction every 6 to 12 weeks. After treatment completion, a 36-month duration monitoring is warranted to confirm recovery. Low-dose methotrexate and azathioprine are the most useful immunosuppressive drugs and are indicated in patients with of failure or contra-indication of corticosteroids or in those patients who needs a long term prednisone dose higher than 10 mg daily to control the sarcoidosis. Cyclophosphamide used is severe neurologic or heart involvement that is resistant to corticosteroids and other immunosuppressive drugs. Hydroxychloroquine and chloroquine are indicated in extensive skin lesions or as corticosteroids sparing agents. Infliximab can be useful in some severe sarcoidosis patients refractory to classical treatment, except for cardiac involvement. Topical corticosteroids can be used for limited skin involvement, anterior uveitis or cough. In the future, a more individualized treatment could be drawn from pharmacogenetic studies. The key point remains the availability of etiologic or innovative drugs for sarcoidosis refractory to currently available therapy.

摘要

结节病是一种严重程度各异的常见疾病。皮质类固醇是其治疗的基石,可使疾病缓解,但仅具有暂时的效果。免疫抑制药物、羟氯喹和英夫利昔单抗可能对某些患者有用。一半的患者未经任何治疗可自发康复。在其余患者中,在疾病发作时或随访期间出现病情复发时需要进行治疗。治疗持续时间应至少为12个月。全身治疗的主要指征包括眼科、神经科、心血管、肾脏、喉部受累、严重的肺部表现、冻疮样狼疮和明显的高钙血症。最初,患者应每天服用0.5至1毫克/千克的泼尼松或泼尼松龙,持续6至12周以实现完全缓解,然后每6至12周逐渐减量。治疗完成后,需要进行36个月的监测以确认康复。低剂量甲氨蝶呤和硫唑嘌呤是最有用的免疫抑制药物,适用于皮质类固醇治疗失败或有禁忌证的患者,或那些需要长期每日服用高于10毫克泼尼松剂量来控制结节病的患者。环磷酰胺用于治疗对皮质类固醇和其他免疫抑制药物耐药的严重神经或心脏受累。羟氯喹和氯喹适用于广泛的皮肤病变或作为皮质类固醇节约剂。英夫利昔单抗对一些经典治疗难治的严重结节病患者可能有用,但心脏受累患者除外。局部皮质类固醇可用于局限性皮肤受累、前葡萄膜炎或咳嗽。未来,药物遗传学研究可能会带来更个体化的治疗方法。关键仍然是要有针对目前可用疗法难治的结节病的病因或创新药物。

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