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. The inclusion of a specific category into ICD depends on utility to the different uses of ICD and sufficient evidence that a health condition exists.  

Conditions in ICD-11

The presence or absence of a specific statistical category ("code" or “concept”) depends on the different applied uses of ICD. However, there needs to be sufficient evidence that a health condition exists and can be clearly diagnosed.

Congenital Lyme disease

The concept of “congenital Lyme disease” was removed from ICD-11 in the last stage of editing. This decision was based on a systematic review conducted by the UK NICE[1]. The decision was later also supported by an independent systematic review by Waddell et al² and by the US CDC³ 

Review of proposals and literature

The different proposals in favour of including “congenital Lyme disease” in ICD-11 cited a total of 15 references available in the literature. WHO cross-checked these references, against those cited earlier in the above-mentioned systematic reviews by UK NICE and Waddell et al. Of the 15 references, one referred to two different editions of the same textbook, and two referred to animal studies. And further two had been explicitly excluded from the analyses because of their study design. The remainder (10) had already been included in at least one of the systematic reviews.

WHO Conclusions

At this time, there is no sufficient evidence for the inclusion of the concept of "congenital Lyme disease" in ICD-11. Similarly, there is no evidence for the removal any other categories from ICD-11 relating to Lyme disease.

How could clinicians record Lyme disease in newborns?

A multitude of mechanisms ensure that information can be recorded, in the event that an affected newborn is identified as having Lyme disease. Disease data using ICD classification is always recorded together with the age of the affected person. Code combinations in ICD-11 allow to record and report relevant details about the condition. 

Should future research indicate or provide evidence for “congenital Lyme disease”, a proposal for amendment of ICD could be submitted.

1. https://www.nice.org.uk/guidance/ng95/documents/evidence-review-14 (draft version contains more detail about the review, criteria and methods)
2. Waddell LA, Greig J, Lindsay LR, Hinckley AF, Ogden NH (2018) A systematic review on the impact of gestational Lyme disease in humans on the fetus and newborn. PLoS ONE 13(11): e0207067. https://doi.org/10.1371/journal. pone.0207067
3. https://www.cdc.gov/lyme/resources/brochure/lymediseasebrochure-P.pdf