Symptom and joint mobility progression in the joint hypermobility syndrome (Ehlers-Danlos syndrome, hypermobility type)

Marco Castori, Isabella Sperduti, Claudia Celletti, Filippo Camerota, Paola Grammatico

Research output: Contribution to journalArticle

Abstract

Objective: To evaluate progression of symptoms and joint mobility in the joint hypermobility syndrome (JHS) in order to identify specific disease pictures by age at presentation. Methods: Fifty JHS patients (44 females, 6 males) were evaluated by Beighton score (BS) calculation, and presence/absence and age at onset of 20 key symptoms. Incidence and prevalence rates by age at onset and sex were calculated and compared by chi-square, Fisher's exact test and Mann-Whitney U-test. Relationship between BS and age at examination was evaluated by the Spearman rho correlation. The existence of an age cut-off separating patients with or without a positive BS was analysed by the receiver operating characteristic analysis. Influence of age on the single components of the BS was also investigated. Results: Except for isolated features, the overall clinical presentation was the same between sexes. In the whole sample, statistically significant differences by age at presentation were registered for fatigue, myalgias, muscle cramps, strains/sprains, dislocations, tendon ruptures, tendonitis, gastroesophageal reflux, chronic gastritis, constipation/diarrhoea and abdominal hernias. A clear inverse correlation between age at examination and BS was demonstrated with an age cut-off fixed at 33 years. Among the components of the BS, spine and elbow joints were not significantly influenced by age. Conclusions: This study confirmed the existence of a protean clinical history of JHS which may be exemplified in different phases with distinguishable presentations. The knowledge of the peculiarities of each of them will help the practitioner in recognising and, hopefully, treating this condition.

Original languageEnglish
Pages (from-to)998-1005
Number of pages8
JournalClinical and Experimental Rheumatology
Volume29
Issue number6
Publication statusPublished - 2011

Fingerprint

Joint Instability
Joints
Age of Onset
Abdominal Hernia
Sprains and Strains
Muscle Cramp
Elbow Joint
Tendinopathy
Myalgia
Gastritis
Constipation
Nonparametric Statistics
Gastroesophageal Reflux
ROC Curve
Tendons
Fatigue
Rupture
Diarrhea
Spine
Incidence

Keywords

  • Ehlers-danlos syndrome hypermobility type
  • Evolution
  • Joint hypermobility syndrome
  • Natural history

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Immunology and Allergy

Cite this

Symptom and joint mobility progression in the joint hypermobility syndrome (Ehlers-Danlos syndrome, hypermobility type). / Castori, Marco; Sperduti, Isabella; Celletti, Claudia; Camerota, Filippo; Grammatico, Paola.

In: Clinical and Experimental Rheumatology, Vol. 29, No. 6, 2011, p. 998-1005.

Research output: Contribution to journalArticle

Castori, Marco ; Sperduti, Isabella ; Celletti, Claudia ; Camerota, Filippo ; Grammatico, Paola. / Symptom and joint mobility progression in the joint hypermobility syndrome (Ehlers-Danlos syndrome, hypermobility type). In: Clinical and Experimental Rheumatology. 2011 ; Vol. 29, No. 6. pp. 998-1005.
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