Department I of Internal Medicine, University Hospital of Cologne, Cologne, Germany; German Centre for Infection Research (DZIF) partner site Bonn-Cologne, Cologne, Germany; CECAD Cluster of Excellence, University of Cologne, Cologne, Germany; Clinical Trials Center Cologne, University Hospital of Cologne, Cologne, Germany.
Mycology Reference Laboratory, National Centre for Microbiology, Instituto de Salud Carlos III, Madrid, Spain.
Lancet Infect Dis. 2019 Dec;19(12):e405-e421. doi: 10.1016/S1473-3099(19)30312-3. Epub 2019 Nov 5.
Mucormycosis is a difficult to diagnose rare disease with high morbidity and mortality. Diagnosis is often delayed, and disease tends to progress rapidly. Urgent surgical and medical intervention is lifesaving. Guidance on the complex multidisciplinary management has potential to improve prognosis, but approaches differ between health-care settings. From January, 2018, authors from 33 countries in all United Nations regions analysed the published evidence on mucormycosis management and provided consensus recommendations addressing differences between the regions of the world as part of the "One World One Guideline" initiative of the European Confederation of Medical Mycology (ECMM). Diagnostic management does not differ greatly between world regions. Upon suspicion of mucormycosis appropriate imaging is strongly recommended to document extent of disease and is followed by strongly recommended surgical intervention. First-line treatment with high-dose liposomal amphotericin B is strongly recommended, while intravenous isavuconazole and intravenous or delayed release tablet posaconazole are recommended with moderate strength. Both triazoles are strongly recommended salvage treatments. Amphotericin B deoxycholate is recommended against, because of substantial toxicity, but may be the only option in resource limited settings. Management of mucormycosis depends on recognising disease patterns and on early diagnosis. Limited availability of contemporary treatments burdens patients in low and middle income settings. Areas of uncertainty were identified and future research directions specified.
毛霉病是一种难以诊断的罕见疾病,发病率和死亡率都很高。诊断往往被延误,疾病往往迅速进展。紧急的手术和医疗干预是救命的。关于复杂的多学科管理的指南有可能改善预后,但在不同的医疗保健环境中,方法存在差异。自 2018 年 1 月以来,来自联合国所有地区的 33 个国家的作者分析了关于毛霉病管理的已发表证据,并提供了共识建议,以解决世界不同地区之间的差异,这是欧洲医学真菌学联合会(ECMM)的“一个世界一个指南”倡议的一部分。诊断管理在世界各地区之间没有很大差异。一旦怀疑患有毛霉病,强烈建议进行适当的影像学检查以记录疾病的范围,并随后强烈建议进行手术干预。强烈建议使用高剂量脂质体两性霉素 B 作为一线治疗,同时强烈建议静脉注射伊曲康唑和静脉或延迟释放片剂泊沙康唑作为中度强度的治疗。两种三唑类药物都强烈推荐作为挽救治疗。由于毒性很大,不推荐使用两性霉素 B 脱氧胆酸盐,但在资源有限的环境中可能是唯一的选择。毛霉病的管理取决于识别疾病模式和早期诊断。在中低收入环境中,由于现有治疗方法的局限性,患者负担沉重。确定了不确定的领域,并指定了未来的研究方向。