{Reference Type}: Systematic Review {Title}: Eumycetoma causative agents: A systematic review to inform the World Health Organization priority list of fungal pathogens. {Author}: Clark JE;Kim HY;van de Sande WWJ;McMullan B;Verweij P;Alastruey-Izquierdo A;Chakrabarti A;Harrison TS;Bongomin F;Hay RJ;Oladele R;Heim J;Beyer P;Galas M;Siswanto S;Dagne DA;Roitberg F;Gigante V;Beardsley J;Sati H;Alffenaar JW;Morrissey CO; {Journal}: Med Mycol {Volume}: 62 {Issue}: 6 {Year}: 2024 Jun 27 {Factor}: 3.747 {DOI}: 10.1093/mmy/myae044 {Abstract}: The World Health Organization, in response to the growing burden of fungal disease, established a process to develop a fungal priority pathogens list. This systematic review aimed to evaluate the epidemiology and impact of eumycetoma. PubMed and Web of Science were searched to identify studies published between 1 January 2011 and 19 February 2021. Studies reporting on mortality, inpatient care, complications and sequelae, antifungal susceptibility, risk factors, preventability, annual incidence, global distribution, and emergence during the study time frames were selected. Overall, 14 studies were eligible for inclusion. Morbidity was frequent with moderate to severe impairment of quality of life in 60.3%, amputation in up to 38.5%, and recurrent or long-term disease in 31.8%-73.5% of patients. Potential risk factors included male gender (56.6%-79.6%), younger age (11-30 years; 64%), and farming occupation (62.1%-69.7%). Mycetoma was predominantly reported in Sudan, particularly in central Sudan (37%-76.6% of cases). An annual incidence of 0.1/100 000 persons and 0.32/100  000 persons/decade was reported in the Philippines and Uganda, respectively. In Uganda, a decline in incidence from 3.37 to 0.32/100  000 persons between two consecutive 10-year periods (2000-2009 and 2010-2019) was detected. A community-based, multi-pronged prevention programme was associated with a reduction in amputation rates from 62.8% to 11.9%. With the pre-specified criteria, no studies of antifungal drug susceptibility, mortality, and hospital lengths of stay were identified. Future research should include larger cohort studies, greater drug susceptibility testing, and global surveillance to develop evidence-based treatment guidelines and to determine more accurately the incidence and trends over time.