Joint hypermobility syndrome (JHS)—also known as Ehlers-Danlos type 3–hypermobile type (hEDS)1—is a poorly recognized connective tissue disorder characterized by increased joint laxity that may affect 10% to 25% of the general population.2 Researchers are increasingly recognizing an association between JHS/hEDS and psychiatric symptoms and disorders, specifically anxiety. In this review, we describe the clinical presentation of JHS/hEDS, propose a new “Neuroconnective phenotype” based on the link between anxiety and JHS/hEDS, and discuss factors to consider when treating anxiety in a patient who has JHS/hEDS.
JHS/hEDS: A complex disorder
Although JHS/hEDS is a heritable condition, several factors are known to influence its prevalence and visibility, including age, sex, and ethnicity; the prevalence is higher among younger patients, females, and African Americans.2 Its known basis is the type and distribution pattern of collagen, and one of the key features used to identify this syndrome is greater joint laxity, meaning increased distensibility of the joints in passive movements as well as a hypermobility in active movements.
Although first described by two dermatologists (Edvard Ehlers and Henri-Alexandre Danlos) at the beginning of the 20th century, JHS/hEDS is now considered a multi-systemic condition. Thus, JHS/hEDS includes a wide range of musculoskeletal features, and over the recent years, extra-articular symptoms, such as easy bruising or hypertrophic scarring, have gained recognition.3 Moreover, individuals with JHS/hEDS frequently present with stress-sensitive illnesses, such as fibromyalgia, or chronic fatigue syndrome.4 The Table2,5,6 provides a description of musculoskeletal and extra-articular features of JHS/hEDS.
The link between JHS/hEDS and anxiety
Psychiatric symptoms are being increasingly recognized as a key feature of JHS/hEDS. Our group published the first case control study on the association between JHS/hEDS and anxiety in 1988.7 Additional studies have consistently replicated and confirmed these findings in clinical and nonclinical populations, and in adult and geriatric patients.8-12 Specifically, JHS/hEDS has been associated with a higher frequency and greater intensity of fears, greater anxiety severity and somatic concerns, and higher frequency of the so-called endogenous anxiety disorders.6,13 There also is limited but growing evidence that JHS/hEDS is associated with depressive disorders, eating disorders, and neurodevelopmental disorders as well as alcohol and tobacco misuse.6,8,11,14,15
Moving toward a new phenotype. Whereas there is increasing evidence of somatic comorbidity in several major psychiatric disorders, present psychiatric nosology does not include specific psychiatric illnesses associated with medical conditions other than organic dementias and secondary psychiatric conditions. However, the overwhelming data on clinical comorbidity (both somatic and psychiatric) require new nosologic approaches. Following the accumulated evidence on this topic over the past 30 years, our group described the “Neuroconnective phenotype” (Figure 1) on the basis of the collected genetic, neurophysiological, neuroimaging, and clinical data.6 The core of the phenotype includes the “anxiety-joint laxity” association and has 5 dimensions that allow for minor overlap (somatic symptoms, somatic illnesses, psychopathology, behavioral dimensions, and somatosensory symptoms). Each of the 5 dimensions includes features that may be present at different degrees with individual variations.
Continue to: Biologic hypotheses...