Is Ehlers-Danlos Syndrome rare – or just rarely diagnosed?

Is Ehlers-Danlos Syndrome rare – or just rarely diagnosed? 

Only 5% of Ehlers-Danlos Syndrome (EDS) sufferers are correctly diagnosed*.

I read this article by Jan Groh, EDS patient & support group leader,
An excerpt:
It’s been estimated that EDS affects more than 1 in 5,000 people worldwide. But, experts think that’s conservative. Each year in the US, 650,000* additional sufferers are un-diagnosed or misdiagnosed due to physician oversight or lack of knowledge about EDS. (*Estimate is according to a study in the UK by Dr. Rodney Grahame.) 
This led me to ask, what exactly is the number of people who might be affected with Ehlers Danlos Syndrome Hypermobility Type (H-EDS) in India?

Dr. Marco Castori writes [1]:
The epidemiology of JHS/EDS-HT has not been properly established. Literature defines EDS a rare disease with a cumulative frequency of 1/5,000 [Steinmann et al., 2002], with JHS/EDS-HT, EDS classic, and vascular types being the most common forms of EDS. Nevertheless, clinical practice suggests a much higher prevalence of JHS/EDS-HT with a proposed frequency of 0.75–2% in the general population [Hakim and Sahota, 2006]. Hence, the real prevalence of JHS/EDS-HT is unknown but likely ranges from ~ 1% to 1/5,000. 

With our population of over a billion, and Indians known to having a higher prevalence rate than caucasians, what do you think is the number of people affected in India?

According to the range proposed by Dr. Castori, the number of H-EDS/JHS patients in India is

1,00,00,000 to 2,00,000 (1 Crores to 2 Lakhs) patients in India

According to Dr. Derek Neilson, the incidence of EDS-HT or H-EDS is closer to 1/100 to 1/500 [2] which translates to

1,00,00,000 to 20,00,000 (1 Crores to 20 Lakhs) patients in India

This is not a rare disease.


[1] Castori, Marco, et al. "Re‐writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers–Danlos syndrome, hypermobility type." American Journal of Medical Genetics Part A 161.12 (2013): 2989-3004.