What is the ICD?

The International Classification serves to record and report health and health-related conditions globally. ICD ensures interoperability of digital health data and their comparability. The ICD contains diseases, disorders, health conditions and much more. Including a specific category into ICD depends on utility to the different uses of ICD and sufficient evidence that a health condition exists.    

Does ICD-11 include Rare Diseases?

ICD-11 includes some 5500 rare diseases and their synonyms. The rare diseases are regularly updated, in close collaboration and conceptual identity with Orphanet. The work is also linked to the "WHO Collaborative Global Network 4 Rare Diseases".

Rare diseases are distributed across different chapters following a primarily clinical approach. The main code is selected according to the most severe system involvement or the specialist most likely to be relied on to manage the disease.

How can Rare Diseases be coded with ICD-11?

Every rare disease is identified in the ICD-11 foundation by a unique identifier, the URI.  Some rare diseases have their individual statistical code.

For rare disease documentation, it is recommended to use the URI. In such a way, all rare diseases can be identified individually.

The ICD-11 coding tool (and API) provides the URI and the statistical code. Access tools and software at https://icd.who.int/icdapi

Where can Rare Diseases be looked up in ICD-11?

Looking up any content of ICD-11 can be done best with the ICD-11 browser. Access the browser in languages other than English and other tools at https://icd.who.int.