Ehlers-Danlos
Syndrome type III: a Clinical review of 1606 patients
Bravo JF 1, Wolff C 2.
1 San Juan de Dios Hospital,
2 Department of Internal Medicine,
Objective. Highlight the high frequency of Dysautonomia
(Dys), Osteoporosis (O), and other signs and symptoms
of Ehlers-Danlos Syndrome type III (EDS-III) that for
most authors is the same as Joint Hypermobility
Syndrome (JHS). JHS can be associated to arthritis. The fact that the Beighton score (BSc) is
insufficient and that the
Patients. Since 2002, out of 2007 new patients we found 1606 JHS patients, in our
Rheumatology Clinic. The BSc and the BC were applied
to all patients. Females 78%. Age: range 16 - 87 y/o, average 46. Patients
were grouped by ages, those less than 30 y/o (Group A), those between 30 and 49
y/o (Group B), and those 50 y/o or older (Group C). Statistical analysis:
Fisher’s exact test and MedCalc software
program.
Results. 1606/2007 new patients had JHS (80%). 112/1606 patients had arthritis
(7.2%): RA (37.5%). Psoriatic arthropathy
(18%). LES (16%) and PEP (14%). Group A 267 patients (16.6%); Group B 646 (40.2%); Group C 693
(43.2%). BSc negative: 56.8%. Marfanoids:
14.3%. Light blue sclera: 76.8%: M 56.1%, F 82.6%. Dys
710/1591 (44.6%): M 24.3%, F 50.4%. In M in groups: A 54.4%, B 26.5%, C 11.6%;
In F in groups: A 79.1%, B 62.9%, C 27.2%. BMD done in 803, low BMD (Osteopenia and O): 71.0%. M 76.7%, F
69.7%. In M in groups: A 66.7%, B 68.4%, C 86.8%. In F in groups: A
64.5%, B 62.7%, C 75.4%. O: 22.4. M 21.2%, F 22.7%. O:
in M in groups: A 14.3% B 12.3%, C 30.9%. In F in groups, A
14.5%, B 13.3%, C 30.5%. Hand-head (87%), Elephant paw (81%), Horizontal thumb
(77%), Valgus elbow (74%); Flying bird hand (72%), Typical soft skin (70%), Typical JHS face (53%).
Conclusions: Dysautonomia
was frequent in young F (79%), more frequent in F at all ages. Low BMD was
frequent, even at young ages, including Osteoporosis (14% in both sexes).
Arthritis was seen in 7%. Light blue sclera was frequent in F (81%). Marfanoid habitus (14%). Other frequent diagnostic signs
(>70%) were: Hand holding head; Elephant paw, Horizontal thumb; Valgus elbow; Flying bird hand; and Typical
soft skin. Typical JHS face (53%). As in previous studies, the Beighton score was again negative in 56.8%. This is
important since in some studies, many JHS patients are not included when only
applying the BSc rather than the BC.