Mechanobiology in the Comorbidities of Ehlers Danlos Syndrome

Abstract

Ehlers-Danlos Syndromes (EDSs) are a group of connective tissue disorders, characterized by skin stretchability, joint hypermobility and instability. Mechanically, various tissues from EDS patients exhibit lowered elastic modulus and lowered ultimate strength. This change in mechanics has been associated with EDS symptoms. However, recent evidence points toward a possibility that the comorbidities of EDS could be also associated with reduced tissue stiffness. In this review, we focus on mast cell activation syndrome and impaired wound healing, comorbidities associated with the classical type (cEDS) and the hypermobile type (hEDS), respectively, and discuss potential mechanobiological pathways involved in the comorbidities.

Mast Cell Involvement in the Pathogenesis of Selected Musculoskeletal Diseases

“…The frequency of all types of Ehlers–Danlos syndrome has been estimated to be at a minimum of 1 in 5000 individuals. Among EDS patients, the hypermobility type is believed to account for up to 80–90% of all EDS patients [ 95 , 96 ]. In this subtype, patients experience hypermobile and unstable joints, along with other signs of weakened connective tissues.…”

“…Therefore, symptoms may include flushing, pruritus, urticaria, angioedema, nasal congestion, nasal pruritus, wheezing, throat swelling, headache, diarrhea, abdominal cramping, unexplained arrhythmias, and hypotensive episodes [ 95 , 98 ]. Some studies reported that up to 25% of patients with hEDS also have MCAS [ 96 ]; however, such associations raise several concerns [ 95 ]. In order to understand the attributed links between MC and EDS, it is worth familiarizing oneself with the information that has made researchers lean towards such hypotheses.…”

“…That is why some scientists do not support the MCAS-hEDS association, as there is too a broad spectrum of symptoms attributed to the hEDS, and there is no sufficient number of objective tests for MCADs and limited knowledge about its pathophysiology [ 95 , 96 ]. Also, many published articles were studied in too small a groups, and some questionnaires were not standardized [ 100 ].…”

The value of dynamic elastomeric fabric orthoses in the management of a complex hypermobile Ehlers‐Danlos syndrome patient: A case report

“… 1 , 3 Additional consideration must also be given to the comorbidities that exist alongside the main condition due to its multisystemic nature. These include Postural Tachycardia Syndrome (PoTS), 4 Mast Cell Activation Syndrome (MCAS), 5 gastrointestinal issues, and bladder and bowel problems. 6 These comorbidities can play an important role when it comes to rehabilitation planning.…”

The value of Dynamic Movement Orthoses in the management of a complex Hypermobile Ehlers-Danlos Syndrome patient: a case report

“…Additional consideration must also be given to the comorbidities that exist alongside the main condition due to its multisystemic nature. These include Postural Tachycardia Syndrome (PoTS) [4], Mast Cell Activation Syndrome (MCAS) [5], gastrointestinal issues and bladder and bowel problems [6]. These comorbidities can play an important role when it comes to rehabilitation planning [7].…”