BACKGROUND: Alveolar echinococcosis is a rare but potentially severe parasitic disease caused by the larval stage of Echinococcus multilocularis, endemic in many countries in the northern hemisphere, including Switzerland. While the liver is most commonly affected, other organs can also be involved either by contiguity or haematogenous spread. To date, there is no epidemiological or clinical data on alveolar echinococcosis in the canton of Geneva.
OBJECTIVES: To describe the demographic, epidemiological, clinical and therapeutic characteristics of alveolar echinococcosis in the canton of Geneva between 2010 and 2021.
METHODS: An investigation was conducted among physicians from Geneva University Hospitals (HUG) and the private sector likely to encounter patients diagnosed with alveolar echinococcosis between 2010 and 2021. All patients being treated in the canton of Geneva were included. After obtaining their consent, an epidemiological questionnaire was completed by patients, and a clinical questionnaire by their referring physicians. Demographic, epidemiological and clinical data were entered into REDCap, then extracted and analysed.
RESULTS: Of a total of 27 patients diagnosed with alveolar echinococcosis, 25 were included in the study; one patient did not provide his consent and one patient could not be contacted. The annual incidence of alveolar echinococcosis in the canton of Geneva was calculated at 0.24 cases per 100,000 inhabitants based on the subset (n = 14) domiciled in Geneva. The vast majority of patients (n = 24; 96%) were followed at HUG. The median age of patients was 55 years (range: 17–79) with a slight predominance of women (56%). Reported risk factors for alveolar echinococcosis included owning a vegetable garden (70.8%), often unfenced, practicing composting (69.6%), and owning a dog (58.3%) or a cat (58.3%). Four patients (16%) had an immunosuppressive condition. Only 52% of patients were symptomatic at the time of diagnosis. The liver was affected in most cases (n = 24; 96%), but one patient had a primary splenic location. Surgical resection for curative purposes was performed in 13 patients (52%). All patients received parasitostatic treatment with albendazole, discontinued in 5 patients (20%) due to drug-induced hepatitis. Three patients died (12%), of which two directly related to alveolar echinococcosis.
CONCLUSION: Alveolar echinococcosis, a rare but severe disease, is endemic in the canton of Geneva. The establishment of mandatory reporting of this disease in Switzerland would allow monitoring of its epidemiological evolution. Primary and secondary prevention measures, currently non-existent, could potentially lower the incidence and severity of the disease.
Lachenmayer A, Gebbers D, Gottstein B, Candinas D, Beldi G. Elevated incidence of alveolar echinococcosis in immunocompromised patients. Food Waterborne Parasitol. 2019 May;16:e00060. 10.1016/j.fawpar.2019.e00060
Torgerson PR, Keller K, Magnotta M, Ragland N. The global burden of alveolar echinococcosis. PLoS Negl Trop Dis. 2010 Jun;4(6):e722. 10.1371/journal.pntd.0000722
Wen H, Vuitton L, Tuxun T, Li J, Vuitton DA, Zhang W, et al. Echinococcosis: advances in the 21st Century. Clin Microbiol Rev. 2019 Feb;32(2):e00075-18. 10.1128/CMR.00075-18
Polish LB, Pritt B, Barth TFE, Gottstein B, O’Connell EM, Gibson PC. European haplotype of Echinococcus multilocularis in the United States. N Engl J Med. (November 17th); 387;20, 2022. DOI: 10.1056/NEJMc2210000.
Chauchet A, Grenouillet F, Knapp J, Richou C, Delabrousse E, Dentan C, et al.; FrancEchino Network. Increased incidence and characteristics of alveolar echinococcosis in patients with immunosuppression-associated conditions. Clin Infect Dis. 2014 Oct;59(8):1095–104. 10.1093/cid/ciu520
Autier B, Gottstein B, Millon L, Ramharter M, Gruener B, Bresson-Hadni S, et al. Alveolar echinococcosis in immunocompromised hosts. Clin Microbiol Infect. 2023 May;29(5):593–9. 10.1016/j.cmi.2022.12.010
Schweiger A, Ammann RW, Candinas D, Clavien PA, Eckert J, Gottstein B, et al. Human alveolar echinococcosis after fox population increase, Switzerland. Emerg Infect Dis. 2007 Jun;13(6):878–82. 10.3201/eid1306.061074
Kern P, Wen H, Sato N, Vuitton DA, Gruener B, Shao Y, et al. WHO classification of alveolar echinococcosis: principles and application. Parasitol Int. 2006;55 Suppl:S283–7. 10.1016/j.parint.2005.11.041
Brunetti E, Kern P, Vuitton DA; Writing Panel for the WHO-IWGE. Expert consensus for the diagnosis and treatment of cystic and alveolar echinococcosis in humans. Acta Trop. 2010 Apr;114(1):1–16. 10.1016/j.actatropica.2009.11.001
Bresson-Hadni S; Swiss Echinococcosis Network. Alveolar echinococcosis in Switzerland. Leading Opinions Internal Medicine 2023, Universimed-https://www.universimed.com/ch/epaper
Piarroux M, Piarroux R, Knapp J, Bardonnet K, Dumortier J, Watelet J, et al.; FrancEchino Surveillance Network. Populations at risk for alveolar echinococcosis, France. Emerg Infect Dis. 2013 May;19(5):721–8. 10.3201/eid1905.120867
Houston S, Belga S, Buttenschoen K, Cooper R, Girgis S, Gottstein B, et al. Epidemiological and Clinical Characteristics of Alveolar Echinococcosis: An Emerging Infectious Disease in Alberta, Canada. Am J Trop Med Hyg. 2021 Mar;104(5):1863–9. 10.4269/ajtmh.20-1577
Grüner B, Kern P, Mayer B, Gräter T, Hillenbrad A, Barth TE, et al. Comprehensive diagnosis and treatment of AE: a single center, long- term observational study of 312 patients in Germany. GMS Infect Dis., 2017, DOI: 10.3205/id000027
Wang X, Dai G, Li M, Jia W, Guo Z, Lu J. Prevalence of human alveolar echinococcosis in China: a systematic review and meta-analysis. BMC Public Health. 2020 Jul;20(1):1105. 10.1186/s12889-020-08989-8
European Food Safety Authority (EFSA),Zancanaro G. Annual assessment of Echinococcus multilocularis surveillance reports submitted in 2020 in the context of Commission Delegated Regulation (EU), 2018/772. EFSA J. 2021. Jan.10.2903/j.efsa.2021.6382
Baumann S, Shi R, Liu W, Bao H, Schmidberger J, Kratzer W, et al.; interdisciplinary Echinococcosis Working Group Ulm. Worldwide literature on epidemiology of human alveolar echinococcosis: a systematic review of research published in the twenty-first century. Infection. 2019 Oct;47(5):703–27. 10.1007/s15010-019-01325-2
Bresson-Hadni S, Bellanger AP, Knapp J, Grenouillet F, Blagosklonov O, Millon L, et al. Echinococcose alvéolaire. EMC Hépatol; 2020. 10.1016/S1155-1976(20)42252-1
Conraths FJ, Probst C, Possenti A, Boufana B, Saulle R, La Torre G, et al. Potential risk factors associated with human alveolar echinococcosis: Systematic review and meta-analysis, PLoS Negl Trop Dis., 2017, DOI: 10.1371/journal. pntd.0005801
Kreidl P, Allerberger F, Judmaier G, Auer H, Aspöck H, Hall AJ. Domestic pets as risk factors for alveolar hydatid disease in Austria. Am J Epidemiol. 1998 May;147(10):978–81. 10.1093/oxfordjournals.aje.a009388
Knapp J, Combes B, Umhang G, Aknouche S, Millon L. Could the domestic cat play a significant role in the transmission of Echinococcus multilocularis? A study based on qPCR analysis of cat feces in a rural area in France. Parasite. 2016;23:42. 10.1051/parasite/2016052
Hegglin D, Bontadina F, Gloor S, Romig T, Deplazes P, Kern P. Survey of public knowledge about Echinococcus multilocularis in four European countries: need for proactive information. BMC Public Health. 2008 Jul;8(1):247. 10.1186/1471-2458-8-247
Knapp J, Demonmerot F, Lallemand S, Richou C, Heyd B, Montange D. Registre français de l’échinococcose alvéolaire : 776 patients et 35 ans de recueil de données épidémiologiques et cliniques. CO31. Congrès annuel de l’Association Française pour l’Etude du Foie. 2021.https://afef.asso.fr
Piarroux M, Piarroux R, Giorgi R, Knapp J, Bardonnet K, Sudre B, et al. Clinical features and evolution of alveolar echinococcosis in France from 1982 to 2007: results of a survey in 387 patients. J Hepatol. 2011 Nov;55(5):1025–33. 10.1016/j.jhep.2011.02.018
Yang YR, Ellis M, Sun T, Li Z, Liu X, Vuitton DA, et al. Unique family clustering of human echinococcosis cases in a chinese community. Am J Trop Med Hyg. 2006 Mar;74(3):487–94. doi: https://doi.org/10.4269/ajtmh.2006.74.487
Vuitton DA, Zhang SL, Yang Y, Godot V, Beurton I, Mantion G, et al. Survival strategy of Echinococcus multilocularis in the human host. Parasitol Int. 2006;55 Suppl:S51–5. 10.1016/j.parint.2005.11.007
Deibel A, Meyer Zu Schwabedissen C, Husmann L, Grimm F, Deplazes P, Reiner CS, et al. Characteristics and Clinical Course of Alveolar Echinococcosis in Patients with Immunosuppression-Associated Conditions: A Retrospective Cohort Study. Pathogens. 2022 Apr;11(4):441. 10.3390/ pathogens11040441 doi: https://doi.org/10.3390/pathogens11040441
Marquis B, Demonmerot F, Richou C, Thiéfin G, Millon L, Wallon M, et al.; Swiss Transplant Cohort Study; FrancEchino Network. Alveolar echinococcosis in solid organ transplant recipients: a case series from two national cohorts. Parasite. 2023;30:9. 10.1051/parasite/2023008
Kern P, Bardonnet K, Renner E, Auer H, Pawlowski Z, Ammann RW, et al. European registry: Human AE, Europe, 1982. Emerg Infect Dis. 2000;2003: 10-3201/eid0903.020341.
Faucher JF, Descotes-Genon C, Hoen B, Godard J, Félix S, Aubry S, et al. Hints for control of infection in unique extrahepatic vertebral alveolar echinococcosis. Infection. 2017 Jun;45(3):365–8. 10.1007/s15010-016-0974-z
Hassan E.S, Recall Bias can be a Threat to Retrospective and Prospective Research Designs, Internet J. Epidemiology, 2005, DOI: 10.5580/2732