Quality Of Life and Burden Of Ehlers-Danlos Syndrome (EDS) Type III

Marion Geoffroy¹, Christelle Guillaume¹, Amélie Servettaz¹, Violaine Laurant-Noel¹, Christine Serratrice Sr.², Jacques Serratrice³, Boris Bienvenu⁴ and Roland Jaussaud⁵, ¹Médecine interne, maladies infectieuses, immunologie clinique, Hôpital Robert Debré. CHU de Reims, Reims, France, ²internal medicine, Foundation Hopital Saint Joseph, Marseille, France, ³Hôpital de la Timone, Marseille, France, ⁴Division of Internal Medicine, Centre Hospitalier Régional Universitaire de Caen, Côte de Nacre, Caen, France, Caen, France, ⁵Médecine interne, maladies infectieuses, immunologie clinique, CHU de Reims, REIMS, France

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: Chronic pain, depression, Ehlers-Danlos syndrome, fatigue and quality of life

Session Information

Title: Fibromyalgia, Soft Tissue Disorders and Pain II

Session Type: Abstract Submissions (ACR)

Background/Purpose:

The Ehlers-Danlos syndrome (EDS) is a clinically and genetically heterogeneous group of inherited connective tissue disorders. Hypermobility type EDS is the most common type of EDS, although commonly misdiagnosed variant of EDS. It is also the most difficult type to diagnose due to the lack of specific diagnostic laboratory tests. This type is characterized by marked joint instability, mild cutaneous involvement, chronic pain, fatigue, gastrointestinal, pelvic, neurologic and cognitive dysfunctions. The burden of delayed diagnosis and misdiagnosis, the severity of symptoms may contribute to explain.

Methods:

A questionnaire study was performed among 43 Hypermobility type EDS patients followed beetwen January and June 2013 in our center. Diagnosis was established on both the Villefranche and Brighton criteria. The following dimensions were assessed : fatigue (Fatigue severity scale, FFS), pain (visual analog scale VAS), anxiety (Hamilton score), depression (Beck depression inventory, BDI-II). Quality of life (QoL) was assessed through the French version of Short Form 36 (SF36).

Results:

Thirty five females (81%) and 8 males (19%) were included during the study period (January to June 2013). All patients had an hypermobility assessed by a Beighton score > 5. The mean age of EDS patients was 41 years. The mean age at onset was 18 years. 84% had a fatigue score ≥ 6 (FFS) and 17% reached the maximal score of 7. Of the patients included, 43 (100%) reported having pain. The mean VAS score was 67 mm. Pain was most frequently localized in neck, shoulder, hips, and forearms, and legs. 90 % of patients reported myalgia in trunk, arms and legs. Headhache, abdominal, thoracic and pelvic pains were also frequently reported (> 50%). All the patients reported anxiety. 92 % suffered from major anxiety. Depression affected 54% of patients and a quarter presented a severe depression as depicted by the BDI-II (score ≥ 27). A marked deterioration of QoL was observed in all SF36 domains.

Conclusion:

Hypermobile EDS patients are characterized by fatigue, musculoskeletal pain, anxiety and depression. All of these symptoms are major determinant of disability, which significantly influences the quality of life. Treatment of pain and fatigue should be a prominent aspect of clinical management of EDS in association with rehabilitation therapies.

Disclosure:

M. Geoffroy,
None;

C. Guillaume,
None;

A. Servettaz,
None;

V. Laurant-Noel,
None;

C. Serratrice Sr.,
None;

J. Serratrice,
None;

B. Bienvenu,
None;

R. Jaussaud,
None.

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