Raynaud's phenomenon, digital ulcers and nailfold capillaroscopy

Raynaud's phenomenon (RP) is a common intrusive vasospastic condition, which is associated with significant pain and disability in patients with systemic sclerosis (SSc). In SSc, there is progressive vasculopathy characterised by microangiopathy and large-vessel involvement, which further compounds digital vasospasm. Therefore, patients with SSc can develop persistent (irreversible) tissue ischaemia including digital ulcers (DUs) and critical ischaemia (gangrene). For patients presenting with RP, careful clinical assessment is required to exclude an underlying secondary cause, including SSc. Key investigations are the detection of structural microvascular alterations by non-invasive nailfold capillaroscopy and the presence of SSc-associated autoantibodies. Patient education is mandatory for both RP and DUs and should be delivered by a dedicated multidisciplinary team including the provision of non-pharmacological interventions. There are a wide range of drug therapies used to treat RP and SSc-DU. Surgical intervention is sometimes required for "complicated" RP (e.g. for refractory DU or gangrene). In the present chapter, we shall discuss unmet needs and challenges including novel approaches to both the assessment and treatment of RP and DUs in SSc.