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  Hypermobility Syndrome — New Diagnostic Criteria
It is 14 years since Dr. Anne H. Child penned her oft cited editorial on the subject of joint hypermobility in The Journal1. In the intervening period there has been a veritable explosion
of knowledge about this fascinating condition2, and it is time for another. The feeling at that time that the hypermobility syndrome as defined in 1967 by Kirk, Ansell, and Bywaters3 might represent
a forme fruste of a heritable disorder of connective tissue (HDCT) has gained momentum and achieved wider acceptance4.
Further clinical studies have helped to define the phenotype and, above all, to emphasize its benign nature in terms of life-threatening complications5. To reflect this, the term “benign
joint hypermobility syndrome” (BJHS) has found increasing popularity. Over the same period clinic and population based studies have confirmed the presence of a higher than previously suspected
prevalence rate of, respectively, the BJHS in rheumatology clinics6 and of joint hypermobility among populations7.
Contrary to previous belief, it has now become well established that pauciarticular or localized hypermobility is more highly prevalent in populations than the more generalized variety8-10. Such
advances in knowledge have seriously brought into question the use of the Beighton 9 point scoring system11 as the sole criterion for diagnosis of hypermobility in the context of the BJHS. It
certainly casts doubt on the logic of selecting an arbitrary Beighton score, be it 5 or 3 (as was the wont in the past), and deciding on that basis whether a patient has the BJHS. Using the Kirk,
et al 1967 definition3, in theory, any symptomatic hypermobile joint (even if it is the only hypermobile joint that patient has) qualifies for the diagnosis of BJHS in that patient’s case.
One of the striking features of the BJHS is the degree of clinical overlap between it and other HDCT, including the marfanoid habitus12, skin hyperextensibility, and a tendency to osteopenia5. This
being so, there is considerable scope for exploiting the presence or otherwise of these extraarticular features in formulating a new approach to the diagnosis of BJHS, one that does not rely solely
on counting the number of hypermobile joints among those from a restricted number of selected sites.
In this issue of The Journal, Grahame, et al offer a validated set of criteria for the classification of the BJHS, termed the the Revised (Brighton 1998) Criteria for the Diagnosis
of BJHS13, which now replaces the provisional set first published in 199214, and takes its place alongside the recently published revised criteria for the Marfan15 and Ehlers-Danlos16 syndromes. The
authors hope that clinicians will find them helpful in distinguishing the BJHS from other phenotypically related conditions, and that they will aid geneticists embarking on molecular genetic studies
in the area of BJHS by clarifying the phenotype of patients under investigation. It remains to be seen whether these lofty aspirations are fulfilled.
JOHN BAUM, MD;
LARS-GÖRAN LARSSON, MD, PhD;
Department of Medicine,
Clinical Immunology and Rheumatology Unit,
University of Rochester,
601 Elmwood Avenue, Box 695,
Rochester, NY 14642, USA.
REFERENCES
1.Child A. Joint hypermobility syndrome: inherited disorder of collagen synthesis. J Rheumatol 1986;13:239-42.
2.Beighton PH, Grahame R, Bird HA. Hypermobility of joints. 3rd ed. London, Berlin, New York: Springer-Verlag; 1999.
3.Kirk JH, Ansell B, Bywaters EGL. The hypermobility syndrome. Musculoskeletal complaints associated with generalized joint
hypermobility. Ann Rheum Dis 1967;26:419-25.
4.Grahame R. Joint hypermobility and genetic collagen disorders. Arch Dis Child 1999;80:188-91.
5.Mishra MB, Ryan P, Atkinson P, et al. Extra-articular features of benign joint hypermobility syndrome. Br J Rheumatol
1996;35:861-6.
6.Bridges AJ, Smith E, Reid J. Joint hypermobility in adults referred to rheumatology clinics. Ann Rheum Dis
1992;51:793-6.
7.Birrell FN, Adebajo AO, Hazleman BL, Silman AJ. High prevalence of joint laxity in West Africans. Br J Rheumatol
1994;33:56-9.
8.Verhoeven J, Tuinma M, Van Dongen WJ. Joint hypermobility in African non-pregnant nulliparous women. Eur J Obstet Gynecol
Reprod Biol 1999;82:69-72.
9.Larsson L-G, Baum J, Muldolkar G, Srivastrava DK. Hypermobility: prevalence and features in a Swedish population. Br J
Rheumatol 1993;32:116-9.
10.Larsson L-G, Baum J, Mudholkar GS. Hypermobility: Features and differential incidence between the sexes. Arthritis Rheum
1987;30:1426-30.
11.Beighton PH, Solomon L, Soskolne CL. Articular mobility in an African population. Ann Rheum Dis 1973;32:413-7.
12.Grahame R, Edwards JC, Pitcher D, Gabell A, Harvey W. A clinical and echocardiographic study of patients with the
hypermobility syndrome. Ann Rheum Dis 1981;40:541-6.
13.Grahame R, Bird HA, Child A, et al. The British Society Special Interest Group on Heritable Disorders of Connective
Tissue Criteria for the Benign Joint Hypermobility Syndrome. The revised (Brighton 1998) criteria for the diagnosis of the BJHS.
J Rheumatol 2000;27:1777-9.
14.Bird HA. Joint hypermobility. Br J Rheumatol 1992;31:205-6.
15.De Paepe A, Devereux RB, Dietz HC, Hennekam RC, Pyeritz RE. Revised diagnostic criteria for the Marfan syndrome. Am J Med
Genet 1996;62:417-26.
16. Beighton P, De Paepe A, Steinmann B, Tsipouras P, Wenstrup RJ. Ehlers-Danlos syndromes: revised nosology, Villefranche,
1997. Am J Med Genet 1998;77:31-7.
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