Annals ofthe Rheumatic Diseases 1992; 51: 793-796 793

Joint hypermobility in adults referred to Ann Rheum Dis: first published as 10.1136/ard.51.6.793 on 1 June 1992. Downloaded from clinics

Alan J Bridges, Elaine Smith, John Reid

Abstract rheumatologist for musculoskeletal problems, hypermobility is a rarely recognised we evaluated 130 consecutive new patients for aetiology for focal or diffuse musculoskeletal joint hypermobility and associated clinical symptoms. To assess the occurrence and features. importance of joint hypermobility in adult patients referred to a rheumatologist, we prospectively evaluated 130 consecutive Patients and methods new patients for joint hypermobility. Twenty PATIENTS women (15%) had joint hypermobility at One hundred and thirty consecutive adult three or more locations (¢5 points on a patients (age >18 years) referred to the out- 9 point scale). Most patients with joint patient rheumatology clinic at the University hypermobility had common musculoskeletal of Missouri-Columbia for musculoskeletal problems as the reason for referral. Two problems or disease were patients referredwith adiagnosis ofrheumatoid evaluated by ES and AJB. There were 97 arthritis were correctly reassigned a diagnosis women and 33 men with an average age of 51 of hypermobility syndrome. Three patients years (range 18-83). with systemic erythematosus had diffuse joint hypermobility. There was a statistically significant association between METHODS diffuse joint hypermobility and . A complete history and physical examination Most patients (65%) had first degree family was performed including an examination for members with a history of joint hypermobility. joint laxity. The criteria devised by Carter and These results show that joint hypermobility is Wilkinson'5 with a modification by Beighton common, familial, found in association with et al 8 were used to assess hypermobility (table common rheumatic disorders, and statistically 1). A mobility score was calculated by allocating

associated with osteoarthritis. The findings one pointfor the ability to perform the manoeuvre http://ard.bmj.com/ support the hypothesis that joint hypermobility at each joint. Two points were allocated if predisposes to musculoskeletal disorders, hypermobility was detectable at bilateral . especially osteoarthritis. Truncal hypermobility received a score of 1. A mobility score greater than or equal to 5 was considered consistent with joint hypermobility. A commonly unrecognised aetiology for focal The following features were also assessed: (a)

or diffuse musculoskeletal problems is the easy bruising; (b) history of joint dislocations; on September 25, 2021 by guest. Protected copyright. hypermobility syndrome. ' 2 The hypermobility (c) history of recurrent joint ; (d) history syndrome refers to a constellation of musculo- or physical finding of joint swelling (effusion); skeletal symptoms and clinical findings of (e) loose skin or hernias; (f) temporomandibular Department of diffuse joint laxity in an otherwise normal and pain; (g) scoliosis; (h) mitral valve Medicine, subject.' 2 When not associated with musculo- prolapse by auscultation (click-murmur with Division of skeletal problems the clinical findings are best characteristic changes on Valsalva manoeuvre) Immunology and Rheumatology, termed diffuse joint hypermobility. Although or echocardiography; (i) passive hyperextension University of the clinical features may mimic inflammatory of one or more proximal interphalangeal joints Missouri School arthritis with joint swelling and pain, the course to greater than 900; (j) passive internal rotation of Medicine, Columbia, MO, USA of joint hypermobility is not progressive, of the hips greater than 450; (k) passive dorsi- A J Bridges destructive, nor disabling.27 flexion of the ankle beyond 150; (I) flat medial E Smith In population studies joint hypermobility has arch of the foot on standing. Department of been found more often in children, with The criteria used to make the diagnoses were Biostatistics, University of diminishing occurrence as age increases.89 Missouri School Joint hypermobility was commonly noted in of Medicine, paediatric patients referred to a rheumatologist Columbia, MO, USA and in normal schoolchildren.' Investigations J Reid in adults have suggested that joint hypermobility Table I Criteria used to evaluate joint hypermobility. Correspondence to: Based on Carter and Wilkinson'" modified by Beighton et Dr Alan Bridges, is associated with osteoarthritis, mitral valve al 8 MA-427 Health prolapse, and chondrocalcinosis.' '3 Only one Sciences Center, Passive dorsiflexion of fifth finger beyond 90° at One Hospital Drive, report has focused on the hypermobility syn- metacarpophalangeal joint University of Missouri drome as a diagnosis in adult patients referred 2 Passive apposition of thumb to flexor aspect of forearm Hospitals and Clinics, 3 Hyperextension of elbow beyond 10° Columbia, MO 65212, USA. to a rheumatologist.'4 To assess the occurrence, 4 Hyperextension of knee beyond 10° modes of and associations of joint 5 Forward flexion of the trunk with the knees fully extended so Accepted for publication presentation, that the palms of the hands rest flat on the floor 8 October 1991 hypermobility in adult patients referred to a 794 Bridges, Smith, Reid

based on the accepted criteria of the American graphy in five patients. Easy bruising (three College of Rheumatology where applicable."6 patients), temporomandibular joint dysfunction Patients with findings including dislocation (two patients), history of joint effusions (five Ann Rheum Dis: first published as 10.1136/ard.51.6.793 on 1 June 1992. Downloaded from of the lens (or positive family history), aortic patients), and recurrent sprains (three patients), regurgitation or family history of aortic rupture, were rare. Joint dislocations, loose skin, and arachnodactyly, excessive arm span, marked hernias were not noted in any patient. skin hyperextensibility, or cigarette paper scars A primary diagnosis of hypermobility were suspected to have Marfan or Ehler-Danlos syndrome was made in two young women with syndrome and not considered to have benign joint mobility scores of 9, recurrent joint hypermobility syndrome. effusions, scoliosis, and . The two patients were referred with a diagnosis of . One patient also had STATISTICS temporomandibular joint dysfunction and Categorical variables (two way tables) were chronic low from sacroiliac hyper- tested using x2 analysis. Continuous variables mobility that became symptomatic during her were tested using the Wilcoxon signed rank or first pregnancy. Neither patient had eye, aortic, Mann-Whitney U tests. skin, or other skeletal abnormalities suggestive of Marfan or Ehlers-Danlos syndrome. Table 2 gives the primary diagnosis for the Results main symptom of each patient with joint hyper- Joint hypermobility was found in 20 (15%) mobility. Causes for the main symptom other patients. Table 2 summarises the findings. The than joint hypermobility were found in 18/20 average age of the patients with joint hyper- patients. Osteoarthritis and were mobility was 50 years (range 23-80), whereas commonly noted in the patients with joint patients without joint hypermobility had an hypermobility. When all diagnoses were taken average age of 52 years (range 18-83); (p>0.4). into consideration, osteoarthritis was found in The patients with joint hypermobility had an 12/20 (60%) patients with joint hypermobility. average mobility score of 8 whereas patients In contrast, 33/110 (30%) patients without joint without joint hypermobility had mobility scores hypermobility had osteoarthritis (X2=6 73; of -2 (average 0-4; p<0 001). All patients with p<0-01). The age of the patients with osteo- joint hypermobility were women whereas only arthritis and joint hypermobility did not differ 75% of the total number of referred patients significantly from the age of the patients with were women. Most patients (65%) with joint osteoarthritis without joint hypermobility (60 hypermobility reported a family history of joint and 66 years respectively; p>005). Fibro- hypermobility in at least one first degree was common, but its occurrence did relative. Hypermobility of the trunk was found not differ significantly between the patients in the younger patients with joint hypermobility with (30%) and without joint hypermobility

(average age 37 years) but was not found in (38%) (p>0.05). No other musculoskeletal http://ard.bmj.com/ older patients with joint hypermobility (average problem occurred more often in patients with age 62 years) (p<0 005). joint hypermobility compared with patients Table 2 shows that patients who met the without joint hypermobility. criteria for joint hypermobility had other Joint hypermobility was found in three features of joint laxity. Many patients had patients with systemic lupus erythematosus hypermobility of the fingers (50%), hips (35%), (SLE) and one patient with incomplete lupus rheumatoid and ankles (50%). Eighty five per cent of the erythematosus. A patient with on September 25, 2021 by guest. Protected copyright. patients had and 35% had scoliosis. arthritis had a joint mobility score of 5. This Mitral valve prolapse was found in nine (45%) patient had a history of hypermobility of the patients. This was documented by echocardio- hands and wrists before the onset of rheumatoid

Table 2 Characteristics ofpatients with joint hypermobility Patient Age Criteria for joint hypermobility Other features Diagnosis for No (years) chief symptom* MCP* Thumb Elbow Knee Back PIP* Swan Hip Ankle Scoliosis MVP* Flat feet 1 34 + + + + + + + + + FM 2 80 + + + + - - + OA 3 48 + + + + SLE, FM 4 44 + + ± + + + FM 5 38 + + + + + + + 6 73 + + + - A + OA 7 23 + + + + + + + + + E + OA 8 54 + + + + + + + + + E + SLE, OA 9 76 + + + - - + - A + OA 10 58 + + + + - + + + + OA 11 34 + + + + + + + + + + E + OA 12 38 + + + + + + - + - + A + ILE 13 48 + + + + + + + - + SLE, OA 14 49 + + + + + + + + - + E + MFP 15 57 + + + + + + - + - + FM, OA 16 72 + + + + - - - + + - - + OA, chondrocalcinosis 17 59 + + + + O-- - A, tendonitis 18 31 + + + + + + + + + A + Bursitis, MFP 19 34 + + + - + + + + RA 20 41 + + + + + - - + OA k(+) finding present; (-) finding not present; (MCP) metacarpal phalangeal joint; (PIP) proximal interphalangeal joint; (MVP) mitral valve prolapse; (A) auscultation; (E) echocardiography; (FM) fibromyalgia; (OA) osteoarthritis; (SLE) systemic lupus erythematosus; (ILE) incomplete lupus erythematosus; (MFP) myofascial pain; (RA) rheumatoid arthritis. J7oint hypermobility in adults 795

arthritis. Each of the patients had intermittent Five patients with systemic rheumatic diseases which may have been due to joint had which may have been due to

hypermobility. In two patients these symptoms joint hypermobility and may have been inappro- Ann Rheum Dis: first published as 10.1136/ard.51.6.793 on 1 June 1992. Downloaded from had been treated with corticosteroids with priately treated. Improved recognition of joint partial response. hypermobility could cirumvent these problems. The finding of joint hypermobility in three Discussion patients with SLE and one with incomplete The evaluation of 130 consecutive adults referred lupus erythematosus may represent a new to a rheumatologist for musculoskeletal problems clinical association. Babini et al recently reported showed that joint hypermobility was common, that 10/59 (17%) patients with SLE had joint familial, found in association with musculo- hypermobility.20 They suggested that joint skeletal disorders, and statistically associated hypermobility may be related to ligamentous with osteoarthritis. laxity and instability in association with Previous studies of joint mobility have shown inflammatory arthritis or hyperparathyroidism that mobility decreases with age with a normal secondary to chronic renal failure. None of the score in the adult of 0-2.' 8 17 Joint mobility lupus patients in this study had acute or chronic scores of -5 were found in less than 2% of or renal insufficiency, and each patient randomly selected adults.8 Other studies had a history of hypermobile features since performed in selected populations (musicians childhood (data not shown). The hereditary and gymnasts) showed joint hypermobility to be nature of hypermobility suggested by this study more common, but unfortunately details of age and others32122 may be linked to the genetic relationships were not given.'7 18 In this study, associations of SLE. The association of joint joint hypermobility was fairly common (15%) hypermobility and SLE, however, is more and the patients had a mean age of 50 years. The likely to be related to the occurrence of two patients did not have common occupations, entities which are common in young women. hobbies, or skills. These comparisons suggest This study lends further support to the that joint hypermobility in patients with association of mitral valve prolapse and joint musculoskeletal problems severe enough to be hypermobility.12 23-25 Mitral valve prolapse was referred to a rheumatologist is more common the most common extra-articular finding in than in the general population. Optimally, an patients with joint hypermobility. This associa- age matched control population should be tion strengthens the hypothesis that patients evaluated for comparison. with diffuse joint hypermobility have a general- Grahame reported a diagnostic survey of ised connective tissue abnormality. referrals to a large rheumatology clinic showing In contrast with other reports which emphasise that hypermobility syndrome was diagnosed in the occurrence of hypermobility in younger 3 25% of women.'4 This corresponds closely patients, we found hypermobile joints in four with the 2-06% of our study population of elderly patients.8 9 No patient in this study had women (two of 97) with hypermobility syn- features of Marfan or Ehlers-Danlos syndrome; http://ard.bmj.com/ drome. however, four patients maintained hypermobile Laxity of the joints has been suggested as a joints to the ages of 72, 73, 76, and 80 years. predisposing factor for joint or Joint hypermobility must be considered in injury. '2519 This may account for the high the differential diagnosis of musculoskeletal percentage of subjects (15%) in this study with symptoms in patients of all ages. joint hypermobility among patients referred to a In conclusion, we found joint hypermobility

rheumatologist. Osteoarthritis was particularly in 15% of adult patients referred to a rheuma- on September 25, 2021 by guest. Protected copyright. common in the patients with joint hypermobility tologist. It is important for doctors to recognise in this study, consistent with other reports. 10 11 this problem to ensure correct diagnosis and Although a direct association between joint treatment because joint hypermobility may hypermobility, joint or soft tissue injury, and mimic rheumatic diseases. Further evaluation osteoarthritis cannot be made from this study, of the association between joint hypermobility this relationship warrants further evaluation. and diseases such as osteoarthritis and SLE is The aetiology and pathogenesis of osteoarthritis warranted. is unknown but may be associated with abnormal joint connective tissue and abnormal joint We appreciate the thoughtful review of the manuscript by Drs Sara Walker and James Cassidy. This work was supported in part biomechanics of stress.'3 Hypermobile joints by NIDRR, Research and Training Center H133B80075. may be associated with abnormal 'loose' Grant, connective tissue and abnormal joint bio- mechanics.2 12 3 Thus joint hypermobility may 1 Kirk J A, Ansell B M, Bywaters E G L. The hypermobility be associated with two mechanisms which are syndrome: musculoskeletal complaints associated with generalized joint hypermobility. Ann Rheum Dis 1967; 26: suggested as playing a causative part in the 419-25. pathogenesis of osteoarthritis. 2 Grahame R. The hypermobility syndrome. Ann Rheum Dis 1990; 49: 199-200. Clinical features of joint hypermobility may 3 Child A H. Joint hypermobility syndrome: inherited disorder mimic systemic rheumatic disease.4 6 This of synthesis. J Rheumatol 1986; 13: 239-42. 4 Gedalia A, Person D A, Brewer E J Jr, Giannini E H. study supports this concept. Two patients Hypermobility of the joints in juvenile episodic arthritis/ presented with a diagnosis of rheumatoid arthralgia. I Pediatr 1985; 107: 873-6. 5 Lewkonia R M, Ansell B M. Articular hypermobility arthritis, although further evaluation substan- simulating chronic rheumatic disease. Arch Dis Child 1983; tiated a diagnosis of hypermobility syndrome. 58: 988-92. 6 Biro F, Gewanter H L, Baum J. The hypermobility syn- This study also emphasises the importance of drome. Pediatrics 1983; 72: 701-6. distinguishing joint hypermobility from coexist- 7 Jessee E F, Owen D S Jr, Sagar K B. The benign hypermobile joint syndrome. Arthritis Rheum 1980; 23: ing, well defined systemic rheumatic diseases. 1053-6. 796 Bridges, Smith, Reid

8 Beighton P, Solomon L, Soskolne C L. Articular mobility in teatures and ditterential incidence between the sexes. an African population. Ann Rheum Dis 1973; 32: 413-8. Arthritis Rheum 1987; 30: 1426-30. 9 Wynne-Davies R. Familial joint laxity. Proc R Soc Med 1971; 19 Nicholas J A. Injuries to knee : relationship to 64: 689-90.

looseness and tightness in football players. 7AMA 1970; Ann Rheum Dis: first published as 10.1136/ard.51.6.793 on 1 June 1992. Downloaded from 10 Scott D, Bird H, Wright V. Joint laxity leading to osteo- 212: 2236-9. arthrosis. Rheumatol Rehabil 1979; 18: 167-9. 20 Babini S M, Cocco J A M, Babini J C, de la Sota M, Arturi A, 11 Bird H A, Tribe C R, Bacon P A. Joint hypermobility leading Marcos J C. Atlantoaxial in systemic lupus to osteoarthrosis and chondrocalcinosis. Ann Rheum Dis erythematosus: further evidence of tendinous alterations. 1978; 37: 203-11. Rheumatol 17: 173-7. 12 Handler C E, Child A, Light N D, Dorrance D E. Mitral .7 1990; valve prolapse, aortic compliance, and skin collagen in joint 21 Beighton P H, Horan F T. Dominant inheritance in familial generalized articular hypermobility. 7 Bone Joint Surg hypermobility syndrome. Br Heart J 1985; 54: 501-8. [Bri 13 Grahame R. How often, when and how does joint hyper- 1970; 52: 145-7. mobility lead to osteoarthritis? Br_J Rheumatol 1989; 28: 22 Horan F T, Beighton P H. Recessive inheritance ofgeneralized 320. joint hypermobility. Rheumatol Rehabil 1973; 12: 47-9. 14 Grahame R. Clinical manifestations of the joint hypermobility 23 Grahame R, Edwards J C, Pitcher D, Gabell A, Harvey W. syndrome. Reumatologia (USSR) 1986; 2: 20-4. A clinical and echocardiographic study of patients with the 15 Carter C, Wilkinson J. Persistent joint laxity and congenital hypermobility syndrome. Ann Rheum Dis 1981; 40: 541-6. dislocation of the hip. .7 Bone Joint Surg [Bri 1%4; 46: 24 Pitcher D, Grahame R. Mitral valve prolapse and joint 40-5. hypermobility: evidence for a systemic connective tissue 16 Schumacher H R, ed. Primer on the rheumatic diseases. 1988. abnormality? Ann Rheum Dis 1982; 41: 352-4. 17 Beighton P B, Grahame R, Bird H A. Hypermobility ofjoints. 25 Ondrasik M, Rybar 1, Rus V, Bosak V. joint hypermobility 2nd ed. Berlin: Springer, 1989. in primary mitral valve prolapse patients. Clin Rheumatol 18 Larsson L-G, Baum J, Mudholkar G S. Hypermobility: 1988; 7: 69-73. http://ard.bmj.com/ on September 25, 2021 by guest. Protected copyright.