摘要：目的： 了解生物制剂治疗难治性Felty综合征的疗效和安全性。 方法：我们报道一例对病情病改善抗风湿药无效、长期需用粒细胞集落刺激因子的严重中性粒细胞减少和复发性细菌感染的难治性Felty综合征，应用美罗华治疗有效，中性粒细胞持续保持正常。通过搜索PubMed英文文献，复习了解生物制剂治疗Felty综合征的证据。

结果：目前，用生物制剂治疗难治性Felty综合征仅为个案报道，且仅局限于美罗华和某些抗TNF制剂（恩利、类克和修美乐），汇总所有报道的病例，其中8例患者使用美罗华，治疗一个疗程后的有效率为62.5%（5/8），中性粒细胞绝对值持续增高>1500/mm3,多数患者的炎性指标和其他临床表现得到改善（包括关节炎、复发性感染和全身症状等），平均随访9个月（6-14个月），仅1例患者复发和中性粒细胞再次降低，再次用美罗华治疗后又快速有效，无相关副作用。而抗TNF制剂的经验仅限于6例患者，其中无一例患者得到持续的中性粒细胞增高。

结论：虽然还得不出最终结论，但从所有报道的全球研究分析来看，仅支持美罗华作为难治性Felty综合征治疗的二线药物。

附原文：Abstract OBJECTIVE: To review and summarize the information available on the effectiveness and safety of biological therapies in refractory Felty's syndrome (FS).METHODS: We describe a case of FS with severe neutropenia and recurrent bacterial infections unresponsive to disease-modifying antirheumatic drug treatment and long-term administration with granulocyte colony-stimulating factor, in which treatment with rituximab (RTX) was useful and resulted in a sustained neutrophil response. Current evidence on the use of biological therapies in FS is also analyzed through a systematic review of the English-language literature, based on a PubMed search. RESULTS: Available data on the use of biological therapies in refractory FS are based only on several case reports and are limited to the use of RTX and some anti-tumor necrosis factor α agents (etanercept, infliximab, and adalimumab). Including the case described here, data are available on 8 patients treated with RTX. A sustained increase in the absolute neutrophil count (>1500/mm(3)) was observed in 62.5% (5/8) of these patients after 1 cycle of treatment. In most of them, the hematological response was accompanied by a parallel improvement in biological markers of inflammation and other clinical manifestations of FS (arthritis, recurrent infections, systemic symptoms, etc). After a median follow-up of 9 months (range, 6-14), only 1 of these patients relapsed and neutropenia reappeared; in this patient, retreatment was rapidly effective. No significant adverse events related to RTX therapy were reported. Experience with anti-tumor necrosis factor agents is limited to 6 patients, none of whom presented any sustained increase in neutrophil count.CONCLUSIONS: Although it is not yet possible to make definite recommendations, the global analysis of all cases reported to date only supports the use of RTX as a second-line therapy in patients with refractory FS.

引自：Narváez J, Domingo-Domenech E, Gómez-Vaquero C, López-Vives L, Estrada P, Aparicio M, Martín-Esteve I, Nolla JM.Biological agents in the management of Felty's syndrome: a systematic review.Semin Arthritis Rheum. 2012 Apr;41(5):658-68.