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Lancet Infect Dis. 2019 Dec;19(12):e405-e421. doi: 10.1016/S1473-3099(19)30312-3. Epub 2019 Nov 5.

Global guideline for the diagnosis and management of mucormycosis: an initiative of the European Confederation of Medical Mycology in cooperation with the Mycoses Study Group Education and Research Consortium.

Author information

1
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. Electronic address: oliver.cornely@uk-koeln.de.
2
Mycology Reference Laboratory, National Centre for Microbiology, Instituto de Salud Carlos III, Madrid, Spain.
3
Department I of Internal Medicine, University Hospital of Cologne, Cologne, Germany; CECAD Cluster of Excellence, University of Cologne, Cologne, Germany.
4
Centre for Infectious Diseases and Microbiology Laboratory Services, New South Wales Health Pathology, and the Department of Infectious Diseases, Westmead Hospital, School of Medicine, University of Sydney, Sydney, NSW, Australia.
5
Université Paris-Descartes, Faculté de Médecine, APHP, Hôpital Européen Georges Pompidou, Unité de Parasitologie-Mycologie, Service de Microbiologie, Paris, France.
6
Radiology, Hospital São Lucas da Pontificia Universidade Catolica do Rio Grande do Sul (PUCRS), Escola de Medicina, Porto Alegre, Brazil; Radiology, Universidade Federal de Ciências da Saúde de Porto Alegre (UFCSPA), Porto Alegre, Brazil.
7
Section of Infectious Diseases and Tropical Medicine and Division of Pulmonology, Medical University of Graz, Graz, Austria; Division of Infectious Diseases and Global Public Health, Department of Medicine, University of California San Diego, San Diego, USA.
8
Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark.
9
Department of Microbiology, Immunology and Transplantation, KU Leuven and Clinical Department of Laboratory Medicine and National Reference Center for Mycosis, University Hospitals Leuven, Leuven, Belgium.
10
Infectious Diseases Clinic, Sant'Orsola-Malpighi Hospital, Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy.
11
Divisions of Critical Care and Pulmonology, Department of Medicine, Charlotte Maxeke Johannesburg Academic Hospital and Faculty of Health Sciences University of the Witwatersrand, Johannesburg, South Africa.
12
Infectious Diseases Unit, 3rd Department of Paediatrics, Faculty of Medicine, Aristotle University School of Health Sciences, Thessaloniki, Greece; Hippokration General Hospital, Thessaloniki, Greece.
13
Division of Infectious Diseases, Department of Medicine, Microbiology and Immunology, McGill University, Montreal, Quebec, Canada.
14
Department of General, Visceral and Cancer Surgery, University Hospital of Cologne, Cologne, Germany.
15
Department of Infectious Diseases, Hacettepe University School of Medicine, Ankara, Turkey.
16
Institut Pasteur, National Reference Center for Invasive Mycoses and Antifungals, Department of Mycology, CNRS UMR2000, Parasitology-Mycology Laboratory, Lariboisière, Saint-Louis, Fernand Widal Hospitals, Assistance Publique-Hôpitaux de Paris (AP-HP), Université de Paris, Paris, France.
17
Westerdijk Fungal Biodiversity Institute, Utrecht, The Netherlands; Centre of Expertise in Mycology RadboudUMC/Canisius Wilhelmina Hospital, Nijmegen, The Netherlands; Ministry of Health, Directorate General of Health Services, Ibri, Oman.
18
Department of Medical Microbiology, Hacettepe University School of Medicine, Sıhhiye Ankara, Turkey.
19
Department of Medical Mycology/Invasive Fungi Research Center (IFRC), School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran.
20
Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Infectious Diseases Unit, Tel Aviv Medical Center, Tel- Aviv, Israel.
21
Dermatology Service & Mycology Department, Hospital General de México "Dr. Eduardo Liceaga", Mexico City, Mexico.
22
Infectious Diseases Unit, Istituto Giannina Gaslini Children's Hospital, Genoa, Italy.
23
Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
24
Special Mycology Laboratory, Division of Infectious Diseases, Department of Medicine, Universidade Federal de São Paulo (UNIFESP), São Paulo, Brazil.
25
Department of Epidemiology and Infectious Diseases, Hospital General Dr Manuel Gea González, Mexico City, Mexico; Medical Mycology and Fungal Immunology/Wellcome Trust Strategic Award Program, Aberdeen Fungal Group, University of Aberdeen, King's College, Aberdeen, UK.
26
Oncohematology Clinic, Faculty of Medicine, Comenius University and National Cancer Institute, Bratislava, Slovakia.
27
InfectiousDisease Research Program, Department of Paediatric Hematology/Oncology and Center for Bone Marrow Transplantation, University Children's Hospital Münster, Münster, Germany.
28
Clinical Microbiology and Infectious Diseases, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Instituto de Investigación v Sanitaria Gregorio Marañón, Madrid, Spain; Medicine Department, School of Medicine, Universidad Complutense de Madrid, Madrid, Spain.
29
Diagnostic and Interventional Radiology, Thoracic Clinic, University Hospital Heidelberg, Heidelberg, Germany.
30
Division of Infectious Diseases, Los Angeles Biomedical Research Institute at Harbor-University of California at Los Angeles (UCLA) Medical Center, Torrance, CA, USA.
31
Department of Internal Medicine, Division of Infectious Diseases, American University of Beirut Medical Center, Beirut, Lebanon.
32
Department of Clinical Mycology, Allergology and Immunology, North Western State Medical University, St Petersburg, Russia.
33
Division of Hygiene and Medical Microbiology, Department of Hygiene, Microbiology and Public Health, Medical University Innsbruck, Innsbruck, Austria.
34
Infectious Diseases Service, Department of Medicine and Institute of Microbiology, Lausanne University Hospital, Lausanne, Switzerland; Institute of Microbiology, Department of Laboratories, Lausanne University Hospital, Lausanne, Switzerland.
35
Institut Pasteur, National Reference Center for Invasive Mycoses and Antifungals, Department of Mycology, Paris Descartes University, Necker-Enfants Malades University Hospital, Department of Infectious Diseases and Tropical Medicine, Centre d'Infectiologie Necker-Pasteur, Institut Imagine, AP-HP, Paris, France.
36
Division of Infectious Diseases, Department of Internal Medicine, Catholic Hematology Hospital, College of Medicine, The Catholic University of Korea, Seocho-gu, Seoul, Korea.
37
Division of Paediatric Haematology and Oncology, Hospital for Children and Adolescents, Johann Wolfgang Goethe-University, Frankfurt, Germany.
38
School of Medicine and Pharmacy, University Mohammed the fifth, Hay Riad, Rabat, Morocco.
39
Laboratory of Antimicrobial Chemotherapy, Ion Ionescu de la Brad University, Iaşi, Romania.
40
Department of Hematology, Oncology and Palliative Care, Klinikum Ernst von Bergmann, Potsdam, Germany.
41
Department of Medical Microbiology and Infectious Diseases, Centre of Expertise in Mycology Radboudumc/Canisius Wilhelmina Hospital, Nijmegen, Netherlands.
42
Clinical Microbiology Laboratory, Attikon University Hospital, National and Kapodistrian University of Athens, Athens, Greece; Department of Medical Microbiology and Infectious Diseases, Erasmus Medical Center, Rotterdam, The Netherlands.
43
Department of Infectious Diseases, Alfred Health & Monash University, Melbourne, Australia.
44
Department of Internal Medicine, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil.
45
Department of Medical Microbiology & Parasitology, College of Medicine, University of Lagos, Yaba, Lagos, Nigeria; Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK.
46
Department of Hematology, Fondazione Policlinico Universitario A. Gemelli -IRCCS- Universita Cattolica del Sacro Cuore, Roma, Italy.
47
Federal University of Health Sciences of Porto Alegre, Hospital Dom Vicente Scherer, Porto Alegre, Brazil.
48
Infectious Diseases Clinic, Vedanta Institute of Medical Sciences, Navarangpura, Ahmeddabad, India.
49
Institute of Hematology and Blood Transfusion, Prague, Czech Republic.
50
UK NHS Mycology Reference Centre, Manchester University NHS Foundation Trust, Manchester, UK.
51
Hämatologie & Internistische Onkologie, Lukas-Krankenhaus Bünde, Onkologische Ambulanz, Bünde, Germany.
52
Department of Medical Mycology/Invasive Fungi Research Center (IFRC), School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran; Center of Expertise in Microbiology, Infection Biology and Antimicrobial Pharmacology, Tehran, Iran; Molecular Microbiology Section, Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
53
Department of Hemato Oncology, Amrita Institute of Medical Sciences, Amrita Viswa Vidyapeetham University, Kochi, India.
54
Division of Infectious Diseases, University of Pittsburgh Medical Center and VA Pittsburgh Healthcare System, Infectious Diseases Section, University of Pittsburgh, Pittsburgh, PA, USA.
55
Infectious Diseases Unit, Szent Istvan and Szent Laszlo Hospital, Budapest, Hungary.
56
Department of Infectious Diseases, Laiko General Hospital, National and Kapodistrian University of Athens, Athens, Greece.
57
University of Melbourne, Melbourne, VIC, Australia; The National Centre for Infections in Cancer, Peter MacCallum Cancer Centre, Parkville, Melbourne, VIC, Australia.
58
P D Hinduja Hospital & Medical Research Centre, Department of Medicine, Veer Sarvarkar Marg, Mumbai, India.
59
Los Angeles County and University of Southern California (LAC+USC) Medical Center, Los Angeles, CA, USA.
60
Division of Pediatric Infectious Diseases, Department of Pediatrics, Duke University Medical Center, Durham, NC, USA.
61
Department of Infectious Diseases, Singapore General Hospital, Singapur, Singapore.
62
Department for Internal Medicine II, University Hospital Würzburg, Würzburg, Germany.
63
Department I of Internal Medicine, University Hospital of Cologne, Cologne, Germany; German Centre for Infection Research (DZIF) partner site Bonn-Cologne, Cologne, Germany; Department of Internal Medicine, Hematology/Oncology, Goethe University Frankfurt, Frankfurt, Germany.
64
Department I of Internal Medicine, University Hospital of Cologne, Cologne, Germany; German Centre for Infection Research (DZIF) partner site Bonn-Cologne, Cologne, Germany; Department of Internal Medicine, Infectious Diseases, Goethe University Frankfurt, Frankfurt, Germany.
65
Departments of Medicine, Pediatrics, Microbiology & Immunology, Weill Cornell Medicine, and New York Presbyterian Hospital, New York City, NY, USA.
66
Public Health Wales Microbiology Cardiff, UHW, Heath Park, Cardiff, UK.
67
Fungus Testing Laboratory, University of Texas Health Science Center, San Antonio, TX, USA.
68
Division of Infectious Diseases, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
69
Department of Medical Microbiology, Postgraduate Institute of Medical Education & Research, Chandigarh, India.

Abstract

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.

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