Background Benign Joint Hypermobility Syndrome (BJHS), is a non-inflammatory condition of generalized joint hypermobility (GJH) associated with musculo-skeletal symptoms such as arthralgia or myalgia in absence of other defined rheumatic diseases. Children referring musculo-skeletal pain (MSP) or growing pain (GP) often present GJH.
Objectives We aimed at clarifying the complexity of the relationship between MSP, GP, GJH and BJHS, which is due to possible overlap of these conditions and to confusing terminology. Our objectives were: 1. To determine the prevalence of GP, MSP, GJH and BJHS in children populations 2. to study the coexistence of these conditions 3. to determine the Beighton’s score (BS) cut-off adopted to indicate GJH.
Methods Literature review and meta-analysis on PubMed database, using the terms “musculoskeletal pain”, “growing pain”, “joint hypermobility” and “benign joint hypermobility syndrome” as inclusion criteria restricted to the age range 0-18 years, were performed. The following sourches have been excluded: comments, letters, editorials, news, reviews, case reports, unavailable rough data, duplicated articles. The analysis was then split in two data collections focusing on: 1. relationship between MSP, GP and GJH as a possible cause 2. relationship between GJH and MSP or GP as possible effect.
Results Of 719 selected articles (years 1972-2011), 37 were considered pertinent.
MSP analysis results (28 articles, 35 cohorts). GP frequency 20.6 - 37% of the general population, 8.2% for patients referred to primary care physicians (PCP), 25.6% in patients with concomitant GJH. MSP frequency 15% - 85% of general population, 6.1% for patients referred to PCP, 74% in patients with concomitant GJH. Pain involved sites: lower limbs 22 – 65.8%; upper limbs 7 - 20%; back 11.4 - 24%.
GHJ analysis results (21 articles, 28 cohorts): GJH frequency ranging between 7,4 (BS cut-off≥6) and 39,4% (BS cut-off≥4) of patients with MSP, 63% among ballet dancers (BS cut-off≥4). MSP frequency: 74% of the GHJ population.
Cut-off of BS chosen to indicate GJH: BS≥4 for 8 studies, BS≥5 for 5 studies, BS≥6 for 4 studies.
Studies using BJHS definition reported a frequency of 4.6 – 9.2% in the general population. According with the data including patients with concomitant MSP/GP and GHJ, satisfying BJHS definition, the frequency of BJHS raises up to 6 – 34% of the general population.
Conclusions The systematic literature review shows an important overlap between MSP, GP, and GJH. Since BJHS has often negative impact on the physical and psychological well-being in pediatric age, its identification becomes essential to correctly address the problem. Therefor, GJH should be always investigated in children with non-inflammatory MSP. A general consensus on BS cut-off is needed in order to better classify patients for clinical research, epidemiology and outcome studies.
Kirk JA, Ansell BM, Bywaters GL, The hypermobility syndrome: musckuloskeletal complaits associated with generalized joint hypermobility. Ann Rheum Dis 1967;26:419-425.
Beighton P, Soloman L, Soskolne CL Articular mobility in an African population. Ann Rheum Dis 1973;32:413-8.
Disclosure of Interest None Declared
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