The first major controversy arises over the value of the diagnostic classification system used to properly diagnose a patient – currently known as the 2017 Diagnostic Criteria for Ehlers-Danlos Syndrome. Depending on who you ask, you will discover if they find value in the method or not, and therein lies the controversy. Many patients feel it is not expansive enough to consider multisystemic aspects of joint hypermobility and question the overall integrity as outlined with typical questions by the community by Chronic Pain Partners. Still, providers say they are happy to at least have a starting point for clinical settings combined with physical exams using the diagnostic checklist published by the EDS Society. They share a GP Toolkit to aid doctors in initial diagnosis by clinical assessment for HSD and hEDS.

The 2017 diagnostic criteria for Ehlers-Danlos Syndromes (EDS) were developed and published by the International EDS Consortium, representing a major revision and update from previous guidelines. This overhaul was motivated by several key factors:

• Increasing Complexity and New Discoveries: Prior to 2017, the last major classification of EDS was in 1997. In the intervening 20 years, significant advancements were made in the understanding of the genetic and clinical aspects of EDS. New types were identified and the molecular basis of various EDS subtypes became clearer. This new knowledge necessitated a reevaluation of the diagnostic approach.
• Clinical Overlap and Misdiagnosis: EDS types often overlap clinically with other heritable connective tissue disorders. The older criteria were sometimes too broad or nonspecific, leading to misdiagnoses or confusion with other conditions such as Marfan syndrome or Loeys-Dietz syndrome. More stringent and specific criteria were needed to improve diagnostic accuracy and ensure appropriate management and care.
• Lack of Consensus: The growing international collaboration among EDS experts highlighted inconsistencies in how the disorders were diagnosed across different regions and specialties. A unified, consensus-driven approach was necessary to standardize the diagnosis globally, ensuring that patients received consistent and accurate evaluations regardless of where they were treated.
• Need for Better Patient Management: Accurate diagnosis is crucial for effective management of EDS, a condition that can significantly impact quality of life. Improved criteria would help clinicians develop better, more tailored treatment plans for patients, addressing the wide array of symptoms and complications that can arise.

Thus, the 2017 criteria were established to address these challenges, providing a comprehensive, updated framework that reflects the latest scientific and clinical insights. This included new classifications for some types, updated descriptions of old types, and the introduction of formal diagnostic criteria for the hypermobile type of EDS, which had long been under-recognized and poorly understood.

Detailed information on the development and specifics of the 2017 criteria

Per the original publication by the International EDS Consortium can be accessed for an in-depth understanding of the methodologies and evidence that shaped these guidelines summarized below. The 2017 International Classification for Ehlers-Danlos Syndromes (EDS) revised the diagnostic criteria for this group of connective tissue disorders, making it more stringent and specific to better differentiate EDS from other hypermobility syndromes such as hypermobility Syndrome Disorder (HSD). These criteria focus on clinical evaluation and family history, aiming to standardize diagnosis across various types of EDS, particularly the hypermobile type (hEDS), which lacks a definitive genetic marker.

The general approach in the 2017 criteria includes:

1. Verification of Generalized Joint Hypermobility (GJH) using tools like the Beighton Score.

2. Presence of systemic features indicative of a broader connective tissue disorder.

3. Family history that suggests an autosomal dominant inheritance pattern.

4. Exclusion of other disorders that could otherwise explain the symptoms.

Specifically, for hEDS, the criteria require a combination of generalized joint hypermobility, systemic manifestations of a connective tissue disorder, and a family history consistent with hEDS. These revised criteria are intended to improve diagnostic accuracy and are particularly detailed for hEDS due to its clinical complexity and lack of a unique genetic test.

Controversy Surrounding the Beighton Score
The EDS Society says its…”range of movement is measured using a goniometer, an instrument that measures the joint angle. A positive Beighton score is any score greater than or equal to 5/9 points in adults, 6/9 points in children (before puberty), and 4/9 points in adults over age 50. To simplify, the Beighton Score is widely used scoring system for assessing generalized joint hypermobility. It scores up to 9 points based on the ability to perform five physical maneuvers, reflecting the flexibility of the joints. A higher score indicates greater hypermobility, with thresholds varying by age and context.

Originally shared as shown here.

Source: Baeza-Velasco, Carolina & Bossche, Thomas & Grossin, Daniel & Hamonet, C.. (2016). Difficulty eating and significant weight loss in Joint Hypermobility Syndrome/ Ehlers–Danlos Syndrome, Hypermobility Type.. Eating and weight disorders: EWD. 21. 175-183. 10.1007/s40519-015-0232-x. 

However, the use of the Beighton Score has been another controversial diagnostic issue due to several concerns:

• Inconsistency in Thresholds: Different medical guidelines and studies use varying cutoffs for what constitutes hypermobility, leading to potential discrepancies in diagnosis.
• Age and Gender Bias: The score does not account for the natural decrease in joint flexibility with age, nor does it adjust for differences typically seen between genders, potentially leading to underdiagnosis in older adults and males.
• Cultural and Racial Differences: There is evidence suggesting that joint mobility can vary significantly across different racial and ethnic groups, which is not considered in the Beighton criteria.

Plus recent study into multimorbidity published from the Norris Lab suggests the scaling system is simply not good enough to catch all instances of hypermobility with its limited joints evaluated mostly in the arms and hands. Due to these issues, some experts advocate for the development of more comprehensive and inclusive criteria or adjustments to the Beighton Score to enhance its diagnostic utility and fairness.

The EDS Society has proclaimed a new 2026 review is coming. We shall see.