Rare diseases in ICD11: making rare diseases visible in health information systems through appropriate coding

The overall revision process was carried out with very limited means considering its scope, ambition and strategic significance, and experienced significant hurdles and setbacks. The lack of funding impacted the level of professionalism that could be attained. The contrast between the initially declared goals and the currently foreseen final product is disappointing. In the context of uncertainty around the outcome of the field testing and the potential willingness of countries to adopt this new version, the European Commission Expert Group on Rare Diseases adopted in November 2014 a recommendation for health care coding systems to consider using ORPHA codes in addition to ICD10 codes for rare diseases having no specific ICD10 codes. The Orphanet terminology, classifications and mappings with other terminologies are freely available at www.orphadata.org.