Anakinra Use During Pregnancy in Patients With Cryopyrin-Associated Periodic Syndromes
Anakinra Use During Pregnancy in Patients With Cryopyrin-Associated Periodic Syndromes
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DOI：
10.1002/art.38811
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发表时间：
2014-11-01
影响因子：
13.3
通讯作者：
Stratton, Pamela
中科院分区：
文献类型：
--
作者：
Chang, Zenas;Spong, Catherine Y.;Stratton, Pamela
Objective.描述妊娠过程和结局以及阿那白滞素（一种重组选择性白细胞介素-1受体阻滞剂）在cryopyrin-associated periodic syndrome（CAPS）患者妊娠期间的应用，CAPS包括家族性寒冷自身炎性综合征（FCAS）、Muckle-Wells综合征（MWS）和胎盘发病的多系统炎性疾病（NOMID）。纳入了目前参加自然史方案并已怀孕的CAPS女性。受试者接受了一个结构化的，标准化的采访方面的产妇健康，怀孕，胎儿的结果。回顾性分析患者病历。9名女性（4名FCAS，2名MWS/NOMID重叠，3名NOMID）报告了1-4次妊娠（FCAS女性共15次妊娠，MWS女性3次妊娠，NOMID女性6次妊娠）。6例FCAS女性和3例NOMID或MWS/NOMID重叠女性在患者接受阿那白滞素时分娩。在服用阿那白滞素期间怀孕的妇女中，继续阿那白滞素治疗;维持孕前剂量，但1名妇女在怀双胞胎期间增加剂量。未观察到早产或严重的妊娠并发症。双胎妊娠的一个胎儿有肾发育不全，并在子宫内死亡。基因检测显示，死亡的双胞胎携带与母亲相同的NLRP 3 c.785T>C，p.V262A突变。另一对双胞胎健康且突变阴性。阿那白滞素在妊娠期间持续用于CAPS妇女，并提供显著的持续症状缓解，同时继续预防CAPS的长期后遗症。阿那白滞素耐受性良好。虽然阿那白滞素和肾发育不全之间的因果关系似乎不太可能，但需要进一步的安全性数据。
Objective. To describe the pregnancy course and outcome and the use of anakinra, a recombinant selective interleukin-1 receptor blocker, during pregnancy in patients with cryopyrin-associated periodic syndromes (CAPS), including familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID).Methods. Women with CAPS who were currently enrolled in natural history protocols and had been pregnant were included. Subjects underwent a structured, standardized interview with regard to maternal health, pregnancy, and fetal outcomes. Medical records were reviewed.Results. Nine women (4 with FCAS, 2 with MWS/NOMID overlap, and 3 with NOMID) reported 1-4 pregnancies (a total of 15 pregnancies in women with FCAS, 3 in women with MWS, and 6 in women with NOMID). Six births to women with FCAS and 3 births to women with NOMID or MWS/NOMID overlap occurred while the patients were receiving anakinra. In women who became pregnant while taking anakinra, the anakinra treatment was continued; the prepregnancy dosage was maintained except in the case of 1 woman whose dosage was increased during a pregnancy with twins. No preterm births or serious complications of pregnancy were observed. One fetus of the twin pregnancy had renal agenesis and died in utero. Genetic testing showed that the deceased twin carried the same NLRP3 c.785T>C, p.V262A mutation as the mother. The other twin was healthy and mutation negative.Conclusion. Anakinra was continued during pregnancy in women with CAPS and provided significant, persistent symptom relief while continuing to prevent the long-term sequelae of CAPS. Anakinra was well tolerated. Although a causal relationship between anakinra and renal agenesis seems unlikely, further safety data are needed.