Aguiar M, Marçal N, Mendes A C, Bugalho de Almeida A
Serviço de Pneumologia 1, Hospital de Santa Maria, CHLN, EPE, Lisboa, Portugal.
Rev Port Pneumol. 2011 Mar-Apr;17(2):85-93.
Despite aggressive treatment, sarcoidosis may be debilitating and progressive. The role of tumor necrosis factor (TNF)-a in the genesis of granulomas is ambiguous. It has proven to be critical in the formation and maintenance of granulomatous inflammation and its antagonist, Infliximab, has therefore been used with success in the treatment of patients with sarcoidosis. There are, however, reports of onset of sarcoidosis in patients in treatment for other conditions and that had no outbursts before submission to this therapy. We used Infliximab in the treatment of patients with sarcoidosis who either didn't respond to corticosteroids and other conventional drugs or developed unacceptable side effects to these drugs. The initial dose was 5mg/Kg body weight and subsequent doses were given at weeks 2, 4 and then every other 8 weeks for a total period of one year. We treated ten patients with biopsy proven sarcoidosis, five men and five women, with a mean age of 47.1 years ranging from 28 to 63 years of age. Three patients had severe neurological symptoms, two had hepatic cirrhosis, one had granulomatous inflammation of the lachrymal gland and had already been submitted to many surgeries, one had extensive pulmonary involvement (stage III), one had disfiguring lupus pernio and two presented disabling cutaneous nodules. In four patients the dosage of corticosteroids or other immunosuppressive drugs was suspended, in three the dosage was reduced and in one, corticosteroids were added to the Infliximab therapy. In five of the patients there was a significant improvement. One of the patients with neurological symptoms displayed a complete recovery, while another had significant improvement of vision deficit enabling her to read again. Two patients withdrew from therapy, one due to lack of improvement of neurological symptoms and the other due to the onset of organizing pneumonia spawned by Infliximab. Two patients developed anti-histone antibodies during treatment. Infliximab seems effective in treating patients who are either refractory or develop side effects to a standard regimen of corticosteroids and immunosuppressive agents. These patients, treated with Infliximab, should be under tight surveillance in order to quickly identify possible secondary effects.
尽管进行了积极治疗,结节病仍可能使人衰弱且呈进行性发展。肿瘤坏死因子(TNF)-α在肉芽肿形成中的作用尚不明确。事实证明它在肉芽肿性炎症的形成和维持中起关键作用,因此其拮抗剂英夫利昔单抗已成功用于治疗结节病患者。然而,有报告称,正在接受其他疾病治疗且在接受该疗法之前无病情发作的患者出现了结节病。我们使用英夫利昔单抗治疗对皮质类固醇和其他常规药物无反应或出现不可接受的副作用的结节病患者。初始剂量为5mg/千克体重,随后在第2周、第4周给药,之后每8周给药一次,共给药一年。我们治疗了10例经活检证实为结节病的患者,5名男性和5名女性,平均年龄47.1岁,年龄范围为28至63岁。3例患者有严重神经症状,2例有肝硬化,1例有泪腺肉芽肿性炎症且已接受多次手术,1例有广泛肺部受累(III期),1例有毁容性冻疮样狼疮,2例有导致残疾的皮肤结节。4例患者停用了皮质类固醇或其他免疫抑制药物,3例患者减少了用药剂量,1例患者在英夫利昔单抗治疗中加用了皮质类固醇。5例患者有显著改善。1例有神经症状的患者完全康复,另1例视力缺陷显著改善,能够再次阅读。2例患者退出治疗,1例是因为神经症状无改善,另1例是因为英夫利昔单抗引发了机化性肺炎。2例患者在治疗期间出现了抗组蛋白抗体。英夫利昔单抗似乎对治疗对皮质类固醇和免疫抑制剂标准方案难治或出现副作用的患者有效。这些接受英夫利昔单抗治疗