Raynaud's phenomenon can be either primary (idiopathic) or secondary to underlying disease including systemic sclerosis. Primary Raynaud's phenomenon is very common, affecting approximately 3%-5% of the general population. Although much rarer, systemic sclerosis-related Raynaud's phenomenon can be particularly severe, progressing to digital ulceration in approximately 50% of patients. Raynaud's phenomenon can have a major impact on quality of life. This review has a focus on the systemic sclerosis-related Raynaud's phenomenon (which is the most researched form of Raynaud's phenomenon and probably the most challenging to treat) and on recent advances. Epidemiology (including transition from 'isolated' to systemic sclerosis-related Raynaud's phenomenon), pathogenesis, diagnosis and assessment are discussed, followed by the treatment of both 'uncomplicated' and 'complicated' Raynaud's phenomena (i.e. Raynaud's phenomenon which has progressed to digital ulceration and/or critical ischaemia). Finally, some of the major challenges for the next 5-10 years are highlighted.
Keywords: Raynaud’s phenomenon; Systemic sclerosis; assessment; diagnosis; management.
© The Author(s) 2019.