Clinical features and treatment of alveolar echinococcosis

Human alveolar echinococcosis is caused by the larval stage of Echinococcus multilocularis, occurring in at least 42 countries of the northern hemisphere. Recent studies in Europe and Asia have shown that the endemic area of E. multilocularis is larger than previously known and the parasite has regionally expanded from rural to urban areas. Diagnosis of alveolar echinococcosis is supported by results from imaging studies, histopathology and/or nucleic acid detection, and serology. The present review summarizes current understanding of clinical features, knowledge on appropriate treatment, and discusses ways to improve standards of care.High prevalences of this deadly disease have been discovered in surveys in parts of China. Clinical manifestations, diagnostic tools and the burden of disease were described, and are based on high case numbers. In Europe, excellent tools have been introduced, which improve disease management. Long-term observations in Switzerland provide an optimistic view, as the infection can be well controlled, if patients are cared for in specialized centres. An expert consensus summarizes the current recommendation for diagnosis and treatment of alveolar echinococcosis by the Informal Working Group on Echinococcosis of the WHO.Diagnosis and treatment of alveolar echinococcosis remains a challenge for clinicians. The updated WHO-recommendations aim to support decisions on diagnosis and treatment of alveolar echinococcosis. Anti-infective therapy is the backbone of treatment; surgery should be restricted to patients at an early stage of the disease. For the majority of cases continuous chemoprophylaxis with benzimidazoles is cost-effective and leads to a good quality of life for patients with this chronic disease.