ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 36

Dysautonomia and Osteoporosis in 2300 Ehlers-Danlos Hypermobile Patients, As Defined By the Brighton Criteria

Jaime F. Bravo, Medical School, Universidad de Chile, santiago, Chile

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords:

Session Information

Date: Sunday, November 13, 2016

Title: Fibromyalgia, Soft Tissue Disorders, Regional and Specific Clinical Pain Syndromes - Poster I: Basic Science Focus

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Dysautonomia and Osteoporosis in 2300 EDS type III patients, as defined by the Brighton criteria  

Background/Purpose: To highlight the high frequency of Dysautonomia (Dys) and Osteoporosis in Ehlers-Danlos type III (EDS-III), complications that are very frequent and usually undiagnosed. Identify the percentage of EDS-III that are not hypermobile (negative Beighton Score (BSc) with positive Brighton Criteria (BC)). Stress the need to make the diagnosis using the Brighton Criteria and not relaying only on the Beighton Score .

Methods: We have studied 2300 EDS-III patients. The BSc and the BC were applied to all patients. Age: range 16 – 87, average 44.43. Females 78.7%.  Patients were grouped in < 30 y/o (A), and over 30 y/o (B). Dysautonomia was clinically evaluated in 2283 patients. Densitometries were done in 1060 patients (OMS criteria).  Patients younger than age 16 were excluded since the BC has been validated only for ages 16 and over.

Results: 2300/2868 patients had EDS-III (80.2%). BSc negative: 51.0%.  Dys total group: positive: M 30.14%, F 56.16%; Group A: M 60.8%, F 84.04%; Group B: M 21.6%, F 60.1%.  BMD total group: normal 31.6%, Osteopenia 47.9%, Osteoporosis 20.5%;  Osteopenia: Group A:  M 45.7%, F 50.4%, Group B: M 53.5%, F 48.0%;  Osteoporosis:  Group A: M 8.6%, F 9.8%, Group B: M 20.0%, F 22.9%.

Positive Dysautonomia (1164) in 2283 EDS-III patients evaluated

 Positive Total  Positive Group  n = 1164 Group A  ( < 30 y/o )  n = 382 Group B  ( ≥ 30 y/o )  n =  782
Males 148 30.4  % 65 60.8  % 83 21.6  %
Females 1016 56.2  % 317 84.0  % 699 60.1  %
  1164   382   782  

Bone Mineral Density studied in 1060 EDS-III patients

Total Group Group A  ( < 30 y/o )   n = 89 Group B  ( ≥ 30 y/o )   n =  741
% Males Females Males Females
Normal  BMD            31.6 45.7 50.1 26.5 29.1
Osteopenia 47.9 45.7 40.1 53.5 48.0
Osteoporosis 20.5 8.6 9.8 20.0 22.9

Conclusion: •        EDS-III is extremely frequent in our Clinic (80.2%), referral Center. •        Significant percentage of EDS-III patients are not hypermobile (BSc negative in 51.0%). In some studies, many EDS-III patients are excluded when only applying the BSc rather than the BC, this is our reason for preferring to use the term Ehlers-Danlos type III, instead of Ehlers-Danlos Hypermobile. •        Dysautonomia is very prevalent in young EDS-III females (84%). Low BMD is frequent, even at young ages, including Osteoporosis,  8.6% in males and 9.8% in females.  

Disclosure: J. F. Bravo, None;

To cite this abstract in AMA style:

Bravo JF. Dysautonomia and Osteoporosis in 2300 Ehlers-Danlos Hypermobile Patients, As Defined By the Brighton Criteria [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/dysautonomia-and-osteoporosis-in-2300-ehlers-danlos-hypermobile-patients-as-defined-by-the-brighton-criteria/. Accessed .

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