When Raynaud's phenomenon (RP) occurs secondary to an underlying condition (most importantly to rheumatologists to connective tissue disease), it can be very severe and progress to tissue injury, for example digital ulceration.
Most work on secondary RP has been in systemic sclerosis (SSc)-related RP and this will be the focus of this lecture, in order to exemplify the key principles of management. The main aims are to reduce symptoms and to prevent progression to digital ulceration and/or critical ischaemia. The first step in management is to establish the diagnosis, and decide whether there are any aggravating or triggering factors (e.g. smoking) amenable to intervention. Non-drug therapy, drug therapy and surgical options will all be discussed, with a focus on recent advances. Although calcium channel blockers remain first-line drug therapy for RP, phosphodiesterase inhibitors are being increasingly used. In those patients who progress to digital ulceration and/or critical ischaemia, intravenous prostanoids and (for patients with recurrent digital ulcers) endothelin receptor antagonism should be considered. For those patients refractory to medical management, surgical debridement of ulcers and/or digital sympathectomy may be beneficial. Botulinum toxin (Botox) injections are given in some centres, but further research is required to establish their efficacy. Studies are ongoing investigating new approaches to therapy.
Upon completion of the session, participants should be able to:
1. Describe the general approach to management of secondary RP, including of digital ulceration and critical ischaemia.
2. Discuss the indications for “advanced therapies” (intravenous prostanoids, endothelin receptor antagonism, surgery).
Disclosure of Interest A. L. Herrick Grant/research support from: Actelion, Consultant for: Actelion and Apricus