Colchicine is recommended at low, weight-adjusted doses to improve the response to medical therapy and prevent recurrences.
Tapering of colchicine is not mandatory but may be considered to prevent persistence of symptoms and re currence.
Corticosteroids should be considered as a second option in patients with contraindications and failure of aspirin or NSAIDs because of the risk of favouring the chronic evolution of the disease and promoting drug dependence; in this case they are used with colchicine.
If used, low to moderate doses (i.e. prednisone 0.2–0.5 mg/kg/day or equivalent) should be recommended instead of high doses (i.e. prednisone 1.0 mg/kg/day or equivalent).
The initial dose should be maintained until resolution of symptoms and normalization of CRP, then tapering should be considered.
由于多中心随机对照研究的开展,2004 年之后心包疾病的治疗有了长足进展。基于这些研究,秋水仙碱被推荐为一线治疗药物,在首次发作心包炎或复发心包炎的患者中,秋水仙碱可加强患者对阿司匹林或 NSAID 的反应,提高缓解率,减少心包炎的复发。秋水仙碱使用特定剂量(无需负荷剂量和根据体重调整)以提高患者的依从性。
Classic NSAIDs (ibuprofen, indomethacin) may be considered during the first and second trimesters; most experts prefer high-dose aspirin, since it is regularly used in antiphospholipid syndrome in pregnancy and is moderately effective in the prevention of preeclampsia in highrisk obstetric patients.After gestational week 20, all NSAIDs (except aspirin ≤100 mg/day) can cause constriction of the ductus arteriosus and impair foetal renal function, and they should be withdrawn in any impair foetal renal function, and they should be withdrawn in any case at gestational week 32. The lowest effective doses of prednisone may be used throughout pregnancy and breastfeeding (with supplementation with calcium and vitamin D). Paracetamol is allowed throughout pregnancy and breastfeeding, as are anti-histamine H2 blockers or proton pump inhibitors. During pregnancy,tapering of therapies should be extremely cautious.Normal vaginal delivery is possible and should be considered in the absence of contraindications. lbuprofen,indomethacin,naproxen and prednisone may be considered in women who are breast-feeding.After discontinuation of breastfeeding, gradual tapering of prednisone should be considered, eventually resuming colchicine.
Colchicine is considered contraindicated during pregnancy and breastfeeding, even though no adverse events during pregnancy andfoetal or child development have been reported in women with FMF treated with colchicine during pregnancy and breactfeedin。发生于妊娠期的最常见的心包疾病是心包积液,通常表现为妊娠晚期的良性轻度积液。该时期最常见的需要药物治疗的疾病是心包炎。在妊娠早中期通常使用传统的非甾体抗炎药治疗,妊娠前 20 周患者应首选阿司匹林。妊娠20周后,临床上禁止使用非甾体抗炎药(除阿司匹林≤100mg/d ),因为该类药物的使用会导致胎儿开放性动脉导管的紧缩及影响胎儿肾功能。而秋水仙碱则禁忌使用,尽管一项研究表明,患有地中海热的女性长期使用该药后,在该类患者的生育、妊娠、胎儿或儿童生长发育中无不良事件发生,也不能在妊娠及哺乳期间使用秋水仙素。