Raynaud’s phenomenon (RP) represents a clinical expression of recurrent
vasospasm of the small arteries and arterioles of the acral parts (most commonly
fingers and toes) provoked by cold exposure and emotional stress. Here, the therapeutic
strategies in primary and secondary RP in systemic sclerosis (SSc) are discussed that
are based on the evolving knowledge about different pathogenic pathways of the
peripheral vascular syndrome. The vasospasm in primary RP is reversible while the
secondary SSc-related RP is associated with endothelial injury and subsequent
structural abnormalities that lead to tissue damage. The disbalance between
vasodilators (nitric oxide - NO, prostacycline) and vasoconstrictors (endothelin-1,
angiotensin) is the major consequence of the endothelial injury in secondary SScrelated
RP. Therapeutic options in primary RP patients include administration of oral,
well-tolerated drugs such as herbal extracts from Ginkgo biloba, pentoxifyllin, or
calcium channel blockers. European League Against Rheumatism recommends
standardised therapeutic approach for management of SSc-related RP that includes
administration of dihydropyridine-type calcium channel blockers, fluoxetine,
phosphodiesterase type 5 inhibitors and intravenous iloprost. Other approaches have
been also studied such as inhibition of renin-angiotensin system, statins, botulinum
toxin but currently there is not enough evidence for their use. Scientific knowledge
about mechanisms of action of different drugs corresponding to the underlying
pathogenesis are discussed as well as available experience in RP regarding efficacy and
safety profile. Individualization of therapy with using complex approach and drug
combinations in resistant cases of severe RP and digital ulcers are presented.
Keywords: Angiotensin, Antiplatelet drugs, Aspirin, Bosentan, Endothelin,
Digital ulcers, Fluoxetine, Iloprost, Nitric oxide, Nifedipine, Pathogenesis,
Pentoxifyllin, Phosphodiesterase inhibitors, Prostacyclin, Raynaud’s
phenomenon, Serotonin, Sildenafil, Systemic sclerosis, Treatment.
