Extra-articular features of benign joint hypermobility syndrome.
To define the phenotype of patients with benign joint hypermobility syndrome (BJHS), we studied 58 consecutive patients (mean age 37 yr) presenting to a rheumatology clinic and 30 controls. Patients underwent rheumatological and ophthalmic examination, hypermobility scoring, echocardiography, measurement of bone mineral density (BMD), and skin thickness, elasticity and light transmissibility. The median hypermobility score was 5/9 Beighton and 31/56 Contompasis. Eighteen (31%) patients complained of significant arthralgia. Six (10%) patients and two (7%) controls had mitral valve prolapse (MVP) (chi(2) = 0.27, P = NS). Neither MVP nor aortic diameters showed a correlation with hypermobility score. There was no significant reduction in BMD. There was a significant correlation between hypermobility and light transmissibility of the skin (r = 0.71, P < 0.0001 Contompasis; r = 0.47, P < 0.05 Beighton) and skin stretchiness (r = 0.49, P < 0.05 Contompasis; r = 0.39, P < 0.05 Beighton). On ophthalmic examination, 14 (41%) patients had upper eyelid laxity. Thus, patients with BJHS do not have an increased prevalence of significant cardiac, bone, skin or eye abnormalities, helping differentiate BJHS from other more serious hereditary disorders of connective tissue.
Mishra
M B
MB
Department of Cardiology, Guy's Hospital, London.
Ryan
P
P
Atkinson
P
P
Taylor
H
H
Bell
J
J
Calver
D
D
Fogelman
I
I
Child
A
A
Jackson
G
G
Chambers
J B
JB
Grahame
R
R
D000293:Adolescent; D000328:Adult; D000368:Aged; D015519:Bone Density; D002319:Cardiovascular System / Q000503:physiopathology; D003240:Connective Tissue Diseases / Q000175:diagnosis / Q000235:genetics; D003937:Diagnosis, Differential; D005123:Eye / Q000503:physiopathology; D005260:Female; D006801:Humans; D007593:Joint Instability / Q000175:diagnosis / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012867:Skin / Q000503:physiopathology; D013577:Syndrome