Abstract
While PoTS may be a primary phenomenon, this section deals with PoTS associated with underlying rheumatic conditions which could be either causal or associations. A relationship with joint hypermobility syndromes, chronic fatigue, Fibromyalgia, Chronic Regional Pain Syndromes and a variety of autoantibodies are discussed in this chapter. Joint hypermobility is common in the general population and may be an asset in performing arts and sport. However, joint laxity/weakness occurs in soft tissue structures such as tendons and ligaments because of faulty production or function of structural proteins such as collagen, fibrillin, elastin, and tenascin. This may be associated with greater ease of injury in some and in others, it may be a feature of an underlying syndrome, collectively termed the Heritable Disorders of Connective Tissue of which the hypermobile subtype of Ehlers-Danlos s yndromes is the commonest. Joint Hypermobility Syndromes are present in > 50% of PoTS patients possibly associated with poor vascular wall integrity and small fibre changes. A variety of autoantibodies to components of cardiac muscle and adrenergic vasoactive receptors suggest a possible mechanism by which PoTS may be influenced by the immune system. An association with autoantibodies found in immune mediated conditions strengthens this link. Strategies to influence PoTS associated with these conditions have not been formally tested in large scale studies. Management of joint hypermobility, Hypermobility Spectrum Disorders (HSD) and hypermobile Ehlers Danlos Syndrome (hEDS) requires a thorough assessment and a problem solving, empowerment-based approach. Physiotherapy is often the mainstay of treatment with occupational therapists, podiatry and other mem bers of the multi-disciplinary team providing additional support and input when needed. Educating individuals on how best to manage symptoms, protect their joints and improve fitness alongside ergonomic adaptation and lifestyle modification are key principles. For other associated causes, especially Chronic Fatigue Syndrome, Fibromyalgia and Chronic Regional Pain Syndrome, the best management strategies for PoTS are as yet unknown. The disparate nature of the rheumatological conditions associated with PoTS may suggest common mechanisms, but it is also plausible they reflect distinct causes (Fig. 1). Further work is needed to clarify subsets of PoTS patients who could then have tailored management strategies. The possibility of combined approaches to pain management, hypermobility together with immunosuppression for those who have underlying autoantibodies holds the promise of integrated s trategies for patients to alleviate the symptoms of PoTS.
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