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How can the World Health Organization’s International Classification of Diseases (ICD)-11 change the clinical management of anaphylaxis?

Anaphylaxis is currently recognized as a severe, life-threatening systemic hypersensitivity reaction characterized by rapid onset and the potential to endanger life. Since anaphylaxis is characterized by rapidly developing life-threatening airway and/or breathing and/or circulation problems, the need of rapid management of this condition is well-known. Epinephrine (adrenaline), injected by the intramuscular route, is the medication of choice for the first-aid treatment of anaphylaxis [1–3]. Regional epidemiological data cite anaphylaxis incidence rates ranging from 1.5 to 7.9 per 100 000 person-years in European countries [4] and estimated in at 5.1% (95%CI, 3.4% to 6.8%) in the United States [5]. In all countries, epidemiological and health services research can serve as a baseline for quality improvement, prioritization of anaphylaxis programs, and eventual reduction in morbidity and mortality. However, the global epidemiological morbidity [6] andmortality [7] data for anaphylaxis remain unclear due to the lack of standardized tools for capturing harmonized and accurate data, particularly in the International Classification of Disease (ICD) (Table 1). As an example, we confirmed in 2012, the under notification of anaphylaxis deaths due to difficult coding under the ICD-10 [7] and documented previously [8,9]. The ICD is an international standard diagnostic classification for mortality and morbidity statistics (MMS) maintained by the World Health organization (WHO). Uses include monitoring of the incidence and prevalence of diseases, observing reimbursements and resource allocation trends, and keeping track of safety and quality guidelines. In use by more than 100 countries, the ICD also includes the counting of deaths as well as diseases, injuries, symptoms, reasons for encounter, factors that influence health status, and external causes of disease [10]. The weak identity of disorders in the ICD contributes to the lack of ascertainment and recognition of their importance for health care planning and resource allocation, and prevents clinical research from being performed. Regardingmortality statistics, unless the data have been compiled using the same methods and according to the same standards, comparisons potentially yield misleading results. For these reasons, theWHO issued international instructions on data collection, coding and classification, and statistical presentation of causes of death. Inmost countries, mortality statistics are routinely compiled according to regulations and recommendations adopted by the World Health Assembly [10]. The international mortality coding instructions presuppose that data have been collected with a death certificate conforming to the International form of medical certificate of cause of death. On current death certificates, a limited number of ICD codes are valid as underlying causes of death, and death certificates do not include the word anaphylaxis per se [7]. Anaphylaxis resides in ICD-10 under the nonspecific ‘Chapter XIX Injury, poisoning and certain other consequences of external causes’ which the main references are ‘Anaphylactic shock due to adverse food reaction’ (T78.0) and ‘Anaphylactic shock, unspecified’ (T78.2) (Table 1). Moreover, the term ‘shock’, which means the drop of blood pressure, is one of the anaphylaxis manifestations, however, should not be taken as synonymous or always together. It suggests the need of implementing a severity degree classification for anaphylaxis into the ICD [6], currently implemented for ICD-11. Understanding the specifications of more accurate anaphylaxis morbidity and mortality statistics can support the identification of patients at highest risk, facilitating the implementation of prevention measures. In theory, such data could be used to support national and international public health strategies such as mapping emerging triggers, assessing the safety of marketed products, and establishing the mandatory declaration of most relevant allergens. Besides, these data can support decision-making actions and advocate for a better management of anaphylaxis through the global affordable availability of auto-injectable adrenaline (AAI), as first-line medications in the treatment of this condition. The WHO lists adrenaline as an essential medication for the treatment of anaphylaxis [9]. However, it isnot readily available in the form of auto-injectors in more than 65% of world countries [11] in which best management of patients with anaphylaxis is therefore impossible. The key issues leading to the lack of availability of AAI include cost but also national regulations, lack of regional evidence about the value of epinephrine and the limited accurate data about the epidemiology of anaphylaxis morbidity and mortality data. For these reasons, regional and international allergy academies support the initiatives to narrow these gaps.