How JHS/hEDS is diagnosed
The Beighton criteria are the most common set of criteria used to diagnose JHS/hEDS.29 In 2000, Grahame et al30 developed the Brighton criteria, which include some extra-articular features. The “Hospital del Mar” criteria31 (also known as the “Bulbena criteria”) were obtained after a multivariate analysis of margins from the Beighton criteria and the original set of criteria described by Rotés. They showed consistent indicators of reliability, internal consistency, and better predictive validity.31
Recently, several self-assessment questionnaires have been developed. Specifically, based on the Hakim and Grahame questionnaire,32 our group developed a novel self-assessment questionnaire that includes pictures to facilitate the diagnosis.33
However, despite multiple ways of assessing JHS/hEDS, it remains mostly undiagnosed and untreated. Because of this, a new clinician-administered checklist has been developed,34 although this checklist does not include the psychiatric aspects of the disorder, so clinicians who use this checklist should ensure that the patient receives additional psychiatric assessment.
Transforming the clinical value into specific interventions
Anxiety disorders are chronic, disabling, and represent the 6th leading cause of disability worldwide.35 They have a significant impact due to the high cost of frequent medical evaluations and treatment of the physical components of the disorder.36 As a clinical marker for a homogeneous type of anxiety, JHS/hEDS can provide valuable information about a patient’s complete clinical picture, especially about the somatic aspects of the disorder.
No randomized controlled trials have been conducted to evaluate pharmacotherapy as treatment for JHS/hEDS. In a cohort study, the overall use of psychotropics was significantly higher in patients with JHS/hEDS compared with controls.37 Anxiety symptoms often are treated with antidepressants, and patients with JHS/hEDS are extremely sensitive to adverse effects. Particularly at the beginning of treatment, they may feel uneasy and restless, and have significant gastrointestinal symptoms, which can exacerbate their anxiety symptoms. Because the anticholinergic effects of tertiary tricyclic antidepressants can reduce abdominal pain and improve bowel movements, this class of medication should be considered. The likelihood of success is greater if medications are started at low doses and are titrated extremely slowly.
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