Arbach Olga, Gross Wolfgang L, Gause Angela
Clinic for Rheumatology, University of Luebeck, Luebeck, Germany.
Immunobiology. 2002 Dec;206(5):496-501. doi: 10.1078/0171-2985-00197.
Churg-Strauss-Syndrome (CSS) often takes a mild course and is in many cases treated successfully by glucocorticosteroids (GC) alone. However, there are also several reports demonstrating the necessity of more intensive treatment in life threatening courses with cyclophosphamide and in less severe cases with other immunosuppressive or immunomodulatory drugs like azathioprine, methotrexate or interferon alpha. Relapses of the CSS are detected clinically and serologically and may require cyclophosphamide therapy as well as high-dose GC. We treated 3 cases between 2000 and 2001 that not only experienced a severe relapse (of the heart and the central nervous system) but also proved to be refractory to cyclophosphamide and GC therapy. In the absence of other options we decided to apply TNF-alpha blockers (etanercept in one case and remicade in the two other). This experimental treatment proved to be effective and safe and induced complete remission in one patient and partial remission in the second and at least stopped disease progression in the third. The BVAS 1 markedly improved after additional treatment with TNF-alpha blockers.
变应性肉芽肿性血管炎（CSS）通常病情较轻，在许多情况下仅用糖皮质激素（GC）就能成功治疗。然而，也有几份报告表明，在危及生命的病程中，需要用环磷酰胺进行更强化的治疗；在病情不太严重的情况下，则需要使用其他免疫抑制或免疫调节药物，如硫唑嘌呤、甲氨蝶呤或α干扰素。CSS的复发可通过临床和血清学检测到，可能需要环磷酰胺治疗以及大剂量GC。我们在2000年至2001年期间治疗了3例患者，这些患者不仅经历了严重复发（心脏和中枢神经系统），而且对环磷酰胺和GC治疗均无效。由于没有其他选择，我们决定应用肿瘤坏死因子-α阻滞剂（1例用依那西普，另外2例用英夫利昔单抗）。这种实验性治疗被证明是有效和安全的，1例患者完全缓解，第2例部分缓解，第3例至少阻止了疾病进展。在加用肿瘤坏死因子-α阻滞剂后，疾病活动度评分（BVAS）1显著改善。
