Abstract

Clinical Study of 972 rheumatological patients with

Joint Hypermobility Syndrome (JHS), using the Brighton Criteria.

Jaime F Bravo ¹, Carlos Wolff ², Sergio L. Correa ².

1.- Clinica Arauco Salud and San Juan de Dios Hospital, Santiago, Chile.

2.- Department of Medicine, University of Chile. Santiago, Chile.

Abstract published in Ann Rheum Dis 2007;66 (Suppl II):244

Background. Joint Hypermobility Syndrome (JHS) is one of the Hereditary Diseases of Connective Tissues (HDCT) and for most authors it is the same as Ehlers-Danlos Syndrome type III. It is probably the principal cause of pain in the rheumatology practice, and usually goes undiagnosed. The prevalence worldwide is around 15%, but in Santiago-Chile, we have found it to be 40% (1). It affects not only the musculo-skeletal system, but due to collagen fragility, most tissues are involved, at times with serious consequences (2).

Objectives. To demonstrate the high frequency of JHS, to discuss the frequency of clinical signs and complications that can be associated with the condition, which at times can be serious, and the lack of sensitivity of the Beighton Score (BSc) in the diagnosis of JHS.

Patients and Methods. At an adult Rheumatology Clinic, from a total of 1898 new patients since 2002, the Brighton Criteria (BC) (3) and the BSc were applied in 1200. Dysautonomia (Dys) was studied in 972, BMD in 611 patients and colour of the sclera in 948 patients (light blue). Other causes of Osteoporosis (O), besides JHS, were excluded both clinically and with laboratory tests. The patients were divided into 3 age groups: A) Younger than 30, B) 30 to 49, C) 50 and over.

Results. Of the 1200 patients, 972 had positive BC, thus the frequency of JHS was 81% (JHS Referral Center). Age range was from 12 to 85 years. Females comprised 78% of the patients. The BSc was negative (3/9 or less) in 61% of the 972 positive BC. Evaluating the BSc it was noted that the most mobile joints were the MCP (61%) and touching the floor with the palm of the hands (66%). BC: Skin abnormalities (96%), back pain (71%), arthralgias (68%), tendinitis (59%), joint subluxations (24%), myopia (24%), varicose veins (20%), Marfanoids (15%), hernias (14%). Normal BMD only 29% (M 17%, F 32%); Low BMD (mild, moderate, severe Osteopenia and O) 71% (M 83%, F 68%), of them 22% had Osteoporosis (M 31%, F 20%); Dys 36% (M 18%, F 41%), and blue sclera in 80% of the patients, with a higher frequency in F (84%), than in M (65%). In patients younger than 30 years old (Group A): Normal BMD 33% (M 24%, F 36%); low BMD was seen in 67% (M 76%, F 64%) and from them 19% had O, with equal distribution in both sexes; Dys 57% (M 40%, F 64%).

Conclusions. JHS is frequent in the rheumatological practice, but in Chile a higher incidence was observed. It is a very common cause of musculo-skeletal pain. Low BMD is frequently seen (71%), even at very young ages (67%). Osteoporosis is very prevalent in JHS (22%), especially in males (31%), and worrisome at the younger age group (19%). Dysautonomia is extremely prevalent in women with JHS under age 30 (64%). Light blue sclera is frequent in females and helpful in the diagnosis. As noticed in our previous study (1) the Beighton score was again insufficient for the diagnosis of JHS and for this reason the Brighton Criteria needs to be used routinely to avoid missing the diagnosis of JHS. Once the diagnosis is made, it is necessary to look for the usual signs and complications, especially Osteoporosis and Dysautonomia, so an early and appropriate treatment is started.

References.

1.- Arthritis Rheum 2006; 54 (2): 515-23.

2.- www.reumatologia-dr-bravo.cl

3.- J Rheumatol 2000; 27: 1777-9