Fungiscope – A Global Database for Rare Fungal Infecons M. J. Vehreschild1, A. Hamprecht2, G. Fischer3, S. de Hoog4, J. J. Vehreschild5 and O.A. Cornely1,6 on behalf of The Fungiscope ECMM/ISHAM Working Group

11st Department of Internal Medicine, University of Cologne, Cologne, Germany, 2Institute for Medical Microbiology, Immunology and Hygiene, University of Cologne, Cologne, Germany, 3 Health Authorities of the State of Baden-Württemberg, Stuttgart, Germany, 4CBS Fungal Biodiversity Centre, Institute of the Royal Netherlands Academy of Arts and Sciences (KNAW), Utrecht, the Netherlands, P-808 51st Department of Internal Medicine, University of Cologne, Cologne, Germany, 6 Clinical Trials Centre Cologne, ZKS Köln, BMBF 01KN1106, University of Cologne, Cologne, Germany

www.fungiscope.net Abstract (Updated Version) Background Registraon for ClinicalSurveys.net

Background: • Worldwide increase in the incidence of We are coordinang a global registry rare IFD for cases of rare invasive fungal • Limited clinical data on risk factors, diseases (IFD). Our objecve is to Web-based clinical course, diagnosis and treatment Diagnoscs Search Engine broaden the knowledge on response Documentaon • Creaon of a global network is warranted epidemiology, to determine the clinical paern of disease, to describe and improve diagnosc Paents and Methods procedures and therapeuc Therapeuc Drug Culture/Biopsy Banking regimens, as well as to facilitate Monitoring Stascal Analysis exchange of clinical isolates among • University-based case registry • Collecon of demographic, clinical and the contributors. Figure 1: Project structure Figure 2: Pathogen distribuon, n=268 microbiological data on rare IFD

• Web-based electronic case report form Methods: at www.fungiscope.net Fungiscope™ - A Global Rare Fungal • Inclusion criteria: Cultural, Infecon Registry uses a web-based histopathological, angen, or molecular electronic case form accessible via biologic evidence of IFD www.fungiscope.net. For inclusion in • Exclusion criteria: Infecons due to the registry we require posive Aspergillus spp., Candida spp., cultures or histopathological, angen Cryptococcus neoformans, Pneumocyss or molecular genec evidence of IFD jiroveci and any endemic fungal infecon or colonizaon and the associated clinical symptoms • Case compensaon: 100 € for each and signs of invasive infecon. The evaluable case, 50 € for each isolate data entered onto the registry • Authorship policy: Restricted to those include demographics, underlying centers contribung paents or condions, neutrophil count, translaonal work to the subset concomitant immunosuppressive published medicaons, clinical signs and • Figure 1 shows an overview of the symptoms of IFD, site of infecon, project’s structure diagnosc tests performed, pathogen idenficaon, anfungal Results treatment, surgical procedures performed, response to treatment, • 268 paents with a rare invasive IFD overall survival and aributable Figure 3: Risk factors of four most frequent pathogens registered between ‘06 - ’12 mortality. • 170 (63%) male paents

• Age range: <1-90 years (median: 45 Results: years) Overall, 268 cases have been • Pathogen distribuon shown in fig. 2 completed. Zygomycetes (n=100; • Distribuon of contribung centers: 37%), Fusarium spp. (n=46; 17%), Germany 66 cases, India 66 cases, Czech yeasts (n=40; 15%), and Republic 34 cases, Brazil 18 cases, Demaaceae (n=32; 12%) were the France 17 cases, Italy 17 cases, , Canada most frequently registered 15 cases, Austria 14 cases, Belgium, Croaa, Cuba, Denmark, France, Israel, pathogens. Chemotherapy or the Netherlands, Romania, Slovakia, allogeneic stem cell transplantaon Thailand, Turkey, USA <5 cases each for a haematological malignancy was • Prevalent risk factors (>=10%) of four the most predominant risk factor most frequent pathogens shown in fig. 3 (n=125; 47%), as well as diabetes • Prevalent sites of infecon (>=10%) of mellitus (n=64; 24%), stay at an ICU four most frequent pathogens shown in (n=48; 18%) and chronic renal fig. 4 disease (n=36; 13%). Sites of • Outcome and mortality four most infecon included the lung in 115 frequent pathogens shown in fig. 5 paents (43%), followed by blood stream infecons (n=56; 21%), the Conclusions and Future Goals Figure 4: Sites of infecon for most frequent pathogens sino-nasal region (n=43, 16%) and deep so ssues (n=41; 15%). For • Increasing relevance of rare IFD 146 (54%) paents, a favourable • Efficient method: 268 cases of rare IFD outcome, defined as a complete or from Europe, North America, Asia and paral response to treatment of IFD South America documented. was documented. Overall mortality • 2011 goals: and mortality aributable to IFD was - Compleon of sequencing of all isolates 43% (n=116) and 31% (n=82), - Introducon of an epidemiological respecvely. survey - Improvement of the FungiQuest™ search Conclusion: engine - Publicaon of another clinical The clinical relevance of rare IFD is subset analysis increasing steadily. In a short period Contact Details: of me, a wide variety of cases from Prof. Oliver A. Cornely, MD, FIDSA and Europe, Asia and South America Maria J.G.T. Vehreschild, MD could be documented. Further University Hospital of Cologne Figure 5: Outcome and mortality 1st Department of Internal Medicine invesgators are cordially invited to Kerpener Str. 62, 50937 Cologne, Germany contribute to Fungiscope. Tel.: +49 221 478 6494, Fax: +49 221 478 3611 Supported by unrestricted grants from Astellas Pharma, Gilead Sciences, MSD/Merck and Pfizer Pharma GmbH email: [email protected], [email protected]