Cold hands – strained heart? Advances in the management of Raynaud's phenomenon and pulmonary hypertension

Excerpt

Vasculopathy is an increasingly recognised partner to inflammatory rheumatological disease. Raynaud's phenomenon (RP) is generally considered a benign vascular manifestation. The natural history of systemic sclerosis (SScl), however, suggests that we underestimate the significance of RP in this context. RP occurs in approximately 90% of SScl patients, often decades before the diagnosis is recognised, and is a harbinger of generalised vasculopathy. Pulmonary arterial hypertension (PAH) is another manifestation of this process, which occurs in approximately 12% of SScl patients [1]. PAH accounts for approximately 50% of mortality in limited SScl and accounts for approximately 7% of mortality in diffuse SScl. It is probable that the vasculopathic processes underlying RP and PAH contribute to the familiar pattern of skin, renal and gastrointestinal pathology. The emergence of effective and conveniently administered therapy for PAH increases the importance of diagnosis and monitoring of this complication. Furthermore, since many principles of PAH management translate to the management of RP, this raises the possibility that the generalised vasculopathy of SScl may also be modifiable. …