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Unexpected association between joint hypermobility syndrome/Ehlers–Danlos syndrome hypermobility type and obsessive–compulsive personality disorder

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Abstract

Joint hypermobility syndrome/Ehlers–Danlos syndrome hypermobility type (JHS/EDS-HT) is a largely unrecognized, heritable connective tissue disorder, mainly characterized by joint instability complications, widespread musculoskeletal pain, and minor skin features. In a case–control study, 47 consecutive JHS/EDS-HT patients were investigated for the prevalence of psychiatric disorders and compared to 45 healthy controls in a single center. The psychiatric evaluation consisted of structured clinical interview for DSM-IV criteria by using the SCID-I and the SCID-II. Symptom severity was assessed using the Hamilton Anxiety Rating Scale (HAM-A), the Hamilton Depression Rating Scale (HAM-D), and the Brief Psychiatric Rating Scale (BPRS). The Global Assessment of Functioning Scale (GAF) was used to assess the overall severity of psychological, social, and occupational functions. JHS/EDS-HT patients had significantly higher mean scores for all questionnaires: HAM-A (6.7 vs. 3.8), HAM-D (6.4 vs. 2.7), GAF (75.0 vs. 86.1), and BPRS (27.5 vs. 25.6). The JHS/EDS-HT group had a 4.3 higher risk of being affected by any psychiatric disorder, and in particular, a 5.8 higher risk of having a personality disorder. In particular, 5 JHS/EDS-HT suffered from obsessive–compulsive personality disorder with an observed prevalence rate of 10.6 % (3.6–23.1). Psychiatric assessment of JHS/EDS-HT patients showed an extremely high prevalence of personality disorders (21 %), and of Axis-I disorders (38 %), mostly depressive. This study did not confirm the previously reported increased rate of panic disorders in JHS/EDS-HT.

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References

  1. Grahame R (2010) What is the joint hypermobility syndrome? JHS from the cradle to the grave. In: Hakim AJ, Keer R, Grahame R (eds) Hypermobility, fibromyalgia and chronic pain. Churchill Livingstone Elsevier, Edinburgh, pp 19–34

    Chapter  Google Scholar 

  2. Tinkle BT, Bird HA, Grahame R, Lavallee M, Levy HP, Sillence D (2009) The lack of clinical distinction between the hypermobility type of Ehlers–Danlos syndrome and the joint hypermobility syndrome (a.k.a. hypermobility syndrome). Am J Med Genet A 149A:2368–2370

    Article  PubMed  Google Scholar 

  3. Hakim AJ, Sahota A (2006) Joint hypermobility and skin elasticity: the hereditary disorders of connective tissue. Clin Dermatol 24:521–533

    Article  PubMed  Google Scholar 

  4. Beighton P, De Paepe A, Steinmann B, Tsipouras P, Wenstrup RJ (1998) Ehlers–Danlos syndromes: revised nosology, Villefranche, 1997. Ehlers–Danlos national foundation (USA) and Ehlers–Danlos support group (UK). Am J Med Genet 77(1):31–37

    Article  PubMed  CAS  Google Scholar 

  5. Grahame R, Bird HA, Child A (2000) The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS). J Rheumatol 27:1777–1779

    PubMed  CAS  Google Scholar 

  6. Mayer K, Kennerknecht I, Steinmann B (2013) Clinical utility gene card for Ehlers–Danlos syndrome types I–VII and variants-update 2012. Eur J Hum Genet 21(1). doi:10.1038/ejhg.2012.162

  7. Hakim AJ, Grahame R (2003) Joint hypermobility. Best Pract Res Clin Rheumatol 17:989–1004

    Article  PubMed  Google Scholar 

  8. Castori M, Sperduti I, Celletti C, Camerota F, Grammatico P (2011) Symptom and joint mobility progression in the joint hypermobility syndrome (Ehlers–Danlos syndrome, hypermobility type). Clin Exp Rheumatol 29:998–1005

    PubMed  Google Scholar 

  9. Castori M, Morlino S, Celletti C, Ghibellini G, Bruschini M, Grammatico P, Blundo C, Camerota F (2013) Re-writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers–Danlos syndrome, hypermobility type. Am J Med Genet A (in press)

  10. Lumley MA, Jordan M, Rubenstein R, Tsipouras P, Evans MI (1994) Psychosocial functioning in the Ehlers–Danlos syndrome. Am J Med Genet 53:149–152

    Article  PubMed  CAS  Google Scholar 

  11. Ercolani M, Galvani M, Franchini C, Baracchini F, Chattat R (2008) Benign joint hypermobility syndrome: psychological features and psychopathological symptoms in a sample pain-free at evaluation 1. Percept Mot Skills 107:246–256

    PubMed  Google Scholar 

  12. Baeza-Velasco C, Gely-Nargeot MC, Vilarrasa AB, Fenetrier C, Bravo JF (2011) Association between psychopathological factors and joint hypermobility syndrome in a group of undergraduates from a French university. Int J Psychiatry Med 41:187–201

    Article  PubMed  Google Scholar 

  13. Garcìa Campavo J, Asso E, Alda M, Andres EM, Sobradiel N (2010) Association between joint hypermobility syndrome and panic disorder: a case–control study. Psychosomatics 51:55–61

    Article  Google Scholar 

  14. Garcia-Campavo J, Asso E, Alda M (2011) Joint Hypermobility and anxiety: the state of the art. Curr Psychiatry Rep 13:18–25

    Article  Google Scholar 

  15. Martin-Santos R, Bulbena A, Crippa JA (2010) Anxiety disorders, their relationship to hypermobility and their management. In: Hakim AJ, Keer R, Grahame R (eds) Hypermobility, fibromyalgia and chronic pain. Churchill Livingstone Elsevier, Edinburgh, pp 56–60

    Google Scholar 

  16. Martín-Santos R, Bulbena A, Porta M, Gago J, Molina L, Duró JC (1998) Association between joint hypermobility syndrome and panic disorder. Am J Psychiatry 155(11):1578–1583

    PubMed  Google Scholar 

  17. Bulbena A, Gago J, Pailhez G, Sperry L, Fullana MA, Vilarroya O (2011) Joint hypermobility syndrome is a risk factor trait for anxiety disorders: a 15-year follow-up cohort study. Gen Hosp Psychiatry 33(4):363–370

    Article  PubMed  Google Scholar 

  18. First MB, Spitzer RL, Gibbon M, Williams JB (2000) Structured clinical interview for DSM-IV Axis I disorders, clinical version (SCID-I CV). Florence, Italy: Organizzazioni Speciali. (Italian version by Mazzi F, Morosini P, de Girolamo G, et al.)

  19. First MB, Gibbon M, Spitzer RL, Williams JBW, Benjamin LS (1997) Structured clinical interview for DSM-IV Axis II personality disorders, (SCID-II). American Psychiatric Press Inc., Washington, DC

    Google Scholar 

  20. Hamilton M (1959) The assessment of anxiety states by rating. Br J Med Psychol 32:50–55

    Article  PubMed  CAS  Google Scholar 

  21. Hamilton M (1960) A rating scale for depression. J Neurol Neurosurg Psychiatry 23:56–62

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  22. Overall JE, Gorham DR (1962) The brief psychiatric rating scale. Psychol Rep 10:799–812

    Article  Google Scholar 

  23. Stata Corp. (2009) Stata statistical software: release 11. Stata Corp LP, College Station, TX

    Google Scholar 

  24. Torgersen S, Kringlen E, Cramer V (2001) The prevalence of personality disorders in community sample. Arch Gen Psychiatry 58:590–596

    Article  PubMed  CAS  Google Scholar 

  25. Celletti C, Camerota F (2013) The multifaced and complex hypermobility syndrome (a.k.a. Ehlers–Danlos syndrome hypermobility type: evaluation and management through a rehabilitative approach. La Clin Terapeutica 164:e325–e335

    CAS  Google Scholar 

  26. Millon T (1981) Disorders of personality: DSM-III, Axis II. Wiley, New York

    Google Scholar 

  27. Bulbena A, Pailhez G, Gago J (2004) “Connective tissue” between panic disorder and dysautonomia. Am J Med 116(11):783 author reply 783–4

    Article  PubMed  Google Scholar 

  28. Gazit Y, Nahir AM, Grahame R, Jacob G (2003) Dysautonomia in the hypermobility syndrome. Am J Med 115:33–40

    Article  PubMed  Google Scholar 

  29. Mathias CJ, Low DA, Iodice V, Owens AP et al (2012) Postural tachycardia syndrome-current experience and concepts. Nat Rev Neurol 8:22–34

    Article  CAS  Google Scholar 

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Correspondence to Claudia Celletti.

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Pasquini, M., Celletti, C., Berardelli, I. et al. Unexpected association between joint hypermobility syndrome/Ehlers–Danlos syndrome hypermobility type and obsessive–compulsive personality disorder. Rheumatol Int 34, 631–636 (2014). https://doi.org/10.1007/s00296-013-2901-2

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