Abstract

Three hundred and nineteen UK‐based consultant rheumatologists, members of the British Society for Rheumatology (a response rate of 76%), responded to a questionnaire concerning their perceptions of the (benign joint) hypermobility syndrome (HMS). The questions were wide‐ranging and covered the nature of the condition, its clinic prevalence, criteria for diagnosis, the efficacy of chosen treatments, the impact of the syndrome on affected individuals and the contribution that it makes to the overall burden of rheumatic disease morbidity. Ninety‐two per cent of the respondents believed in the HMS as a distinct clinical entity but only 39% accepted it as a distinct pathological entity. Only 42% were prepared to comment on whether the HMS and Ehlers–Danlos syndrome, hypermobility type (formerly EDS type III) were one and the same entity. There was striking variability in estimated clinic prevalence and no consensus about the diagnostic criteria being used. There was little enthusiasm for treatments currently available. Nearly one‐half of the respondents were sceptical about a significant impact of the HMS on people's lives and three‐quarters about a significant contribution to the overall burden of rheumatic disease. There was little sign of awareness of findings in recent published studies. It seems unlikely, therefore, that evidence‐based medicine is being practised in this area of rheumatology. An unexpected finding was a refreshing enthusiasm for joining regional interest groups on hypermobility (25% of all UK consultants expressed interest).

The subspecialty of rheumatology is not immune to controversy. Broad areas of consensus exist on ideas relating to the pathogenesis, natural history and treatment of such major rheumatic diseases as rheumatoid arthritis and osteoarthritis. By contrast, heated debate rages when topics like fibromyalgia syndrome (FMS) [1] are discussed, advocates and sceptics adhering to fervently held opposing viewpoints on even fundamental aspects of the condition. In 1992 a questionnaire on their perceptions of FMS was sent to a one‐in‐seven sample of all UK‐based members of the British Society for Rheumatology [2]. It was clear that there existed a wide diversity of perception of FMS and differences in the approach to its diagnosis and treatment.

Joint hypermobility is another condition considered to be controversial by many rheumatologists. It also happens to be statistically associated with FMS in both adults [3] and children [4]. The purpose of the present survey was to explore the perceptions held by British rheumatologists on various aspects of the hypermobility syndrome (HMS), a condition originally defined by Kirk et al. in 1967 [5] as the presence of musculoskeletal symptoms (predominantly pain) in hypermobile but otherwise healthy individuals. In recent years, the term ‘benign joint hypermobility syndrome’ (BJHS) has been used more widely [6]. A validated set of diagnostic criteria for the BJHS, known as the 1998 Brighton Criteria, has been published recently [7].

Methods

In early 1999 a questionnaire was sent to all 420 UK‐based consultant rheumatologist members of the British Society for Rheumatology concerning their perceptions of the BJHS. The questions were wide‐ranging and covered the nature of the condition, its perceived clinic prevalence, the criteria used for diagnosis, the efficacy of chosen treatments, the impact of the syndrome on affected individuals and the contribution it makes to the overall burden of rheumatic disease morbidity.

Results

Three hundred and nineteen replies were received and analysed. This corresponds to a response rate of 76%. The questions and an analysis of the responses are shown in Table 1.

Ninety‐two per cent of the respondents believed in the HMS as a distinct clinical entity but only 39% accepted it as a distinct pathological entity. Only 53% were prepared to comment on whether the HMS and Ehlers–Danlos syndrome, hypermobility type (formerly EDS type III) were one and the same entity. There was striking variability in the estimated clinic prevalence and no consensus as to the diagnostic criteria being used. Therapeutic nihilism was widely prevalent, with little enthusiasm for the treatments currently available. Reassurance alone was thought by most to be the most helpful treatment. Nearly one‐half of the respondents were sceptical about the syndrome having a significant impact on people's lives and three‐quarters about a significant contribution to the overall burden of rheumatic disease. Thirty‐five per cent of the respondents indicated an interest in joining a regional interest group on hypermobility. This represents 25% of all UK consultants, and these respondents covered all parts of the UK. Five such groups are currently in the process of being set up. The names and addresses of the regional convenors are shown in Appendix 1.

Table 1.

Questionnaire and responses

Do you believe in HMS as a distinct clinical entity? Yes, 92% No, 7% NR, 1%   
Do you believe in HMS as a pathological entity? Yes, 39% No, 51% NR, 10%   
In your opinion, are HMS and Ehlers–Danlos type 3 (EDS III): One and the same, 9% Different entities, 44% DK, 46% NR, 1%  
Approximately how many HMS cases have you seen in the past year? None, 3%, <10, 48% 11–25, 35% 26–50, 10% >50, 2% DK, 2% 
Which features do you regard as necessary for a diagnosis of HMS? Yes No NR   
   Musculoskeletal symptoms 80% 15% 5%   
   Beighton score ⩾5/9, 58% ⩾3/9, 29% ⩾1/9, 1% >0/9,1% NR, 11% 
   Positive family history 10% 79% 11%   
   Extra‐articular features 6% 84% 10%   
   Negative laboratory findings 61% 30% 9%   
What treatment modalities do you generally recommend, and how effective are they? Very effective Not very effective Useless Do not use NR 
   Reassurance only 56% 35% 1% 3% 5% 
   Physiotherapy 33% 49% 6% 6% 6% 
   Simple analgesics/NSAIDs 14% 69% 6% 5% 6% 
   Surgery 1% 5% 11% 74% 9% 
   Osteopathy/acupuncture/homeopathy 2% 20% 6% 64% 8% 
   Cognitive behavioural therapy 4% 10% 4% 75% 7% 
   Psychiatric referral 1% 3% 7% 82% 7% 
   Other(s) (specify) Advice, 6 Anti‐depressants, 3 Glucosamine SO4, 1 Splints, 1 Information sheet, 1 
 Podiatry, 4 Knee orthosis, 1 Fitness programme, 1 Joint protection, 1 Benign neglect, 1 
 Self‐exercises, 4 Taping patella, 1    
How do you rate the impact of HMS on peoples' lives in most cases? Serious, 1% Significant, 50% Minimal, 45% None, 1% NR, 3% 
What contribution does HMS make to the overall burden of rheumatic disease morbidity? Major, 72% Significant, 24% Minimal, 72% None, 1% NR, 3% 
Would you like to become involved in a regional interest group on HMS? Yes, 104 No, 201 Maybe, 10 NR, 4  
Do you believe in HMS as a distinct clinical entity? Yes, 92% No, 7% NR, 1%   
Do you believe in HMS as a pathological entity? Yes, 39% No, 51% NR, 10%   
In your opinion, are HMS and Ehlers–Danlos type 3 (EDS III): One and the same, 9% Different entities, 44% DK, 46% NR, 1%  
Approximately how many HMS cases have you seen in the past year? None, 3%, <10, 48% 11–25, 35% 26–50, 10% >50, 2% DK, 2% 
Which features do you regard as necessary for a diagnosis of HMS? Yes No NR   
   Musculoskeletal symptoms 80% 15% 5%   
   Beighton score ⩾5/9, 58% ⩾3/9, 29% ⩾1/9, 1% >0/9,1% NR, 11% 
   Positive family history 10% 79% 11%   
   Extra‐articular features 6% 84% 10%   
   Negative laboratory findings 61% 30% 9%   
What treatment modalities do you generally recommend, and how effective are they? Very effective Not very effective Useless Do not use NR 
   Reassurance only 56% 35% 1% 3% 5% 
   Physiotherapy 33% 49% 6% 6% 6% 
   Simple analgesics/NSAIDs 14% 69% 6% 5% 6% 
   Surgery 1% 5% 11% 74% 9% 
   Osteopathy/acupuncture/homeopathy 2% 20% 6% 64% 8% 
   Cognitive behavioural therapy 4% 10% 4% 75% 7% 
   Psychiatric referral 1% 3% 7% 82% 7% 
   Other(s) (specify) Advice, 6 Anti‐depressants, 3 Glucosamine SO4, 1 Splints, 1 Information sheet, 1 
 Podiatry, 4 Knee orthosis, 1 Fitness programme, 1 Joint protection, 1 Benign neglect, 1 
 Self‐exercises, 4 Taping patella, 1    
How do you rate the impact of HMS on peoples' lives in most cases? Serious, 1% Significant, 50% Minimal, 45% None, 1% NR, 3% 
What contribution does HMS make to the overall burden of rheumatic disease morbidity? Major, 72% Significant, 24% Minimal, 72% None, 1% NR, 3% 
Would you like to become involved in a regional interest group on HMS? Yes, 104 No, 201 Maybe, 10 NR, 4  

NR, not recorded; DK, do not know; NSAIDs, non‐steroidal anti‐inflammatory drugs.

Table 1.

Questionnaire and responses

Do you believe in HMS as a distinct clinical entity? Yes, 92% No, 7% NR, 1%   
Do you believe in HMS as a pathological entity? Yes, 39% No, 51% NR, 10%   
In your opinion, are HMS and Ehlers–Danlos type 3 (EDS III): One and the same, 9% Different entities, 44% DK, 46% NR, 1%  
Approximately how many HMS cases have you seen in the past year? None, 3%, <10, 48% 11–25, 35% 26–50, 10% >50, 2% DK, 2% 
Which features do you regard as necessary for a diagnosis of HMS? Yes No NR   
   Musculoskeletal symptoms 80% 15% 5%   
   Beighton score ⩾5/9, 58% ⩾3/9, 29% ⩾1/9, 1% >0/9,1% NR, 11% 
   Positive family history 10% 79% 11%   
   Extra‐articular features 6% 84% 10%   
   Negative laboratory findings 61% 30% 9%   
What treatment modalities do you generally recommend, and how effective are they? Very effective Not very effective Useless Do not use NR 
   Reassurance only 56% 35% 1% 3% 5% 
   Physiotherapy 33% 49% 6% 6% 6% 
   Simple analgesics/NSAIDs 14% 69% 6% 5% 6% 
   Surgery 1% 5% 11% 74% 9% 
   Osteopathy/acupuncture/homeopathy 2% 20% 6% 64% 8% 
   Cognitive behavioural therapy 4% 10% 4% 75% 7% 
   Psychiatric referral 1% 3% 7% 82% 7% 
   Other(s) (specify) Advice, 6 Anti‐depressants, 3 Glucosamine SO4, 1 Splints, 1 Information sheet, 1 
 Podiatry, 4 Knee orthosis, 1 Fitness programme, 1 Joint protection, 1 Benign neglect, 1 
 Self‐exercises, 4 Taping patella, 1    
How do you rate the impact of HMS on peoples' lives in most cases? Serious, 1% Significant, 50% Minimal, 45% None, 1% NR, 3% 
What contribution does HMS make to the overall burden of rheumatic disease morbidity? Major, 72% Significant, 24% Minimal, 72% None, 1% NR, 3% 
Would you like to become involved in a regional interest group on HMS? Yes, 104 No, 201 Maybe, 10 NR, 4  
Do you believe in HMS as a distinct clinical entity? Yes, 92% No, 7% NR, 1%   
Do you believe in HMS as a pathological entity? Yes, 39% No, 51% NR, 10%   
In your opinion, are HMS and Ehlers–Danlos type 3 (EDS III): One and the same, 9% Different entities, 44% DK, 46% NR, 1%  
Approximately how many HMS cases have you seen in the past year? None, 3%, <10, 48% 11–25, 35% 26–50, 10% >50, 2% DK, 2% 
Which features do you regard as necessary for a diagnosis of HMS? Yes No NR   
   Musculoskeletal symptoms 80% 15% 5%   
   Beighton score ⩾5/9, 58% ⩾3/9, 29% ⩾1/9, 1% >0/9,1% NR, 11% 
   Positive family history 10% 79% 11%   
   Extra‐articular features 6% 84% 10%   
   Negative laboratory findings 61% 30% 9%   
What treatment modalities do you generally recommend, and how effective are they? Very effective Not very effective Useless Do not use NR 
   Reassurance only 56% 35% 1% 3% 5% 
   Physiotherapy 33% 49% 6% 6% 6% 
   Simple analgesics/NSAIDs 14% 69% 6% 5% 6% 
   Surgery 1% 5% 11% 74% 9% 
   Osteopathy/acupuncture/homeopathy 2% 20% 6% 64% 8% 
   Cognitive behavioural therapy 4% 10% 4% 75% 7% 
   Psychiatric referral 1% 3% 7% 82% 7% 
   Other(s) (specify) Advice, 6 Anti‐depressants, 3 Glucosamine SO4, 1 Splints, 1 Information sheet, 1 
 Podiatry, 4 Knee orthosis, 1 Fitness programme, 1 Joint protection, 1 Benign neglect, 1 
 Self‐exercises, 4 Taping patella, 1    
How do you rate the impact of HMS on peoples' lives in most cases? Serious, 1% Significant, 50% Minimal, 45% None, 1% NR, 3% 
What contribution does HMS make to the overall burden of rheumatic disease morbidity? Major, 72% Significant, 24% Minimal, 72% None, 1% NR, 3% 
Would you like to become involved in a regional interest group on HMS? Yes, 104 No, 201 Maybe, 10 NR, 4  

NR, not recorded; DK, do not know; NSAIDs, non‐steroidal anti‐inflammatory drugs.

Discussion

This survey of opinion of UK consultant rheumatologists is based on 319 questionnaires returned out of 420 sent out, a 76% response rate. Like its predecessor on fibromyalgia [1], it has uncovered a deeply disturbing picture—one of great diversity of opinion about almost all the aspects probed. While the majority at least acknowledged that the BJHS exists as a clinical entity, only 39% believed that it has a pathological basis. There is compelling evidence that, as seen from the clinic perspective, the BJHS is a forme fruste of a heritable disorder of connective tissue [8]. Yet only 13 respondents accepted the currently widely held view that it is identical with the hypermobility type of the Ehlers–Danlos syndrome (formerly EDS type III). Excluding the handful who denied the very existence of the BJHS, there was a fivefold variation in estimated clinic prevalence. A true difference in clinic prevalence of such proportions is clearly out of the question. The only logical explanation for these perceived differences is the failure on the part of many consultants to recognize the presence of hypermobility in their patients. Joint hypermobility is easy to recognize if one looks for it, but equally easy to overlook if one does not [9]. It would appear that, for many rheumatologists, testing for hypermobility does not yet form part of their routine examination repertoire.

Musculoskeletal pain was a key element in the original definition of the HMS provided by Kirk et al. [5]. It was therefore somewhat surprising to find that 20% of respondents were prepared to make the diagnosis in its absence. The scoring system of Beighton et al. [10] clearly enjoys well‐deserved widespread recognition and use. However, it was designed not for clinical use but for epidemiological studies. Its shortcoming is that it gives no indication of the degree of joint laxity, merely the widespread nature or otherwise of hypermobility in that individual. Epidemiological studies [11, 12] have shown that pauciarticular hypermobility is even more highly prevalent in the community than the more familiar polyarticular variety. Yet in terms of the clinical consequences of hypermobility—pain, instability, dislocation, etc.—which may occur in a single lax joint, it matters not how many other joints may or may not be affected. It is clearly illogical to deny a patient a (correct) diagnosis of the BJHS merely because she does not have a Beighton score of ⩾5 or ⩾3, which is exactly what 87% of respondents say they do (another 11% did not say!). A positive family history and the presence of extra‐articular features are commonly encountered, and it appears that some (but not many) colleagues feel that these are essential for diagnosis. A striking finding was that 61% required a negative laboratory screen before they would be prepared to summon up sufficient courage to make a diagnosis of the BJHS. This smacks of defensive medicine. Is it due to a lack of confidence in one's clinical findings, a need to make such a diagnosis by exclusion rather than by inclusion, or does it signify a lack of conviction in the very existence of the entity itself? It is hoped that the revised 1998 Brighton criteria [7] for the diagnosis of the BJHS will prove to be of assistance to clinicians and researchers.

The respondents' comments on treatment are not surprising. The therapeutic nihilism reflects the genuine difficulty in alleviating the symptoms of the BJHS successfully. The results of conventional methods of treatment, as commonly used in rheumatology, are generally disappointing in the BJHS; alternative, newer strategies are required, and some of these appear to be more effective [13]. The low perception of the impact of the syndrome on individuals' lives or on its contribution to the overall burden of rheumatic disease morbidity is challenged by the patients themselves [14], and is contrary to published findings [15, 16]. An unexpected finding was a refreshing enthusiasm for joining regional interest groups on hypermobility (25% of all UK consultants expressed interest).

This survey confirms previous suspicions that the BJHS is a condition that is under‐recognized and underestimated by rheumatologists. There was little sign of awareness of the findings of recent published studies [34, 69,1116]. It seems unlikely that evidence‐based medicine is being practised in this area of rheumatology.

Appendix 1.

Regional hypermobility special interest groups

Region
 
Convenor
 
Address
 
London and South‐East Professor Rodney Centre for Rheumatology, University College London Hospitals, 
    Grahame    40–50 Tottenham Street, London W1P 9PG 
North‐East and North‐West Professor Howard Bird Chapel Allerton Hospital, Leeds, West Yorkshire LS7 4SA 
Midlands and East Anglia Dr George Struthers Department of Rheumatology, Walsgrove Hospital NHS Trust, 
     Clifford Bridge Road, Coventry CV2 2DX 
Wessex, South‐West and Wales Dr Michael I. D. Cawley Rheumatology Unit, West Wing, Southampton University Hospital, 
     Southampton, Hants SO16 6YD 
Scotland and Northern Ireland Professor Roger Sturrock Centre for Rheumatic Diseases, Royal Infirmary, 10 Alexandra Parade, 
     Glasgow G31 2ER 
Region
 
Convenor
 
Address
 
London and South‐East Professor Rodney Centre for Rheumatology, University College London Hospitals, 
    Grahame    40–50 Tottenham Street, London W1P 9PG 
North‐East and North‐West Professor Howard Bird Chapel Allerton Hospital, Leeds, West Yorkshire LS7 4SA 
Midlands and East Anglia Dr George Struthers Department of Rheumatology, Walsgrove Hospital NHS Trust, 
     Clifford Bridge Road, Coventry CV2 2DX 
Wessex, South‐West and Wales Dr Michael I. D. Cawley Rheumatology Unit, West Wing, Southampton University Hospital, 
     Southampton, Hants SO16 6YD 
Scotland and Northern Ireland Professor Roger Sturrock Centre for Rheumatic Diseases, Royal Infirmary, 10 Alexandra Parade, 
     Glasgow G31 2ER 
Appendix 1.

Regional hypermobility special interest groups

Region
 
Convenor
 
Address
 
London and South‐East Professor Rodney Centre for Rheumatology, University College London Hospitals, 
    Grahame    40–50 Tottenham Street, London W1P 9PG 
North‐East and North‐West Professor Howard Bird Chapel Allerton Hospital, Leeds, West Yorkshire LS7 4SA 
Midlands and East Anglia Dr George Struthers Department of Rheumatology, Walsgrove Hospital NHS Trust, 
     Clifford Bridge Road, Coventry CV2 2DX 
Wessex, South‐West and Wales Dr Michael I. D. Cawley Rheumatology Unit, West Wing, Southampton University Hospital, 
     Southampton, Hants SO16 6YD 
Scotland and Northern Ireland Professor Roger Sturrock Centre for Rheumatic Diseases, Royal Infirmary, 10 Alexandra Parade, 
     Glasgow G31 2ER 
Region
 
Convenor
 
Address
 
London and South‐East Professor Rodney Centre for Rheumatology, University College London Hospitals, 
    Grahame    40–50 Tottenham Street, London W1P 9PG 
North‐East and North‐West Professor Howard Bird Chapel Allerton Hospital, Leeds, West Yorkshire LS7 4SA 
Midlands and East Anglia Dr George Struthers Department of Rheumatology, Walsgrove Hospital NHS Trust, 
     Clifford Bridge Road, Coventry CV2 2DX 
Wessex, South‐West and Wales Dr Michael I. D. Cawley Rheumatology Unit, West Wing, Southampton University Hospital, 
     Southampton, Hants SO16 6YD 
Scotland and Northern Ireland Professor Roger Sturrock Centre for Rheumatic Diseases, Royal Infirmary, 10 Alexandra Parade, 
     Glasgow G31 2ER 

Correspondence to: R. Grahame.

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