title: Raynaud's phenomenon: from molecular pathogenesis to therapy.
abstract: Raynaud's phenomenon (RP) is a well defined clinical syndrome characterized by recurrent episodes of digital vasospasm triggered by exposure to physical/chemical or emotional stress. RP has been classified as primary or secondary, depending on whether it occurs as an isolated condition (pRP) or is associated to an underlying disease, mainly a connective tissue disease (CTD-RP). In both cases, it manifests with unique "triple" (pallor, cyanosis and erythema), or "double" color changes. pRP is usually a benign condition, while sRP can evolve and be complicated by acral digital ulcers and gangrene, which may require surgical treatment. The pathogenesis of RP has not yet been entirely clarified, nor is it known whether autoantibodies have a role in RP. Even so, recent advances in our understanding of the pathophysiology have highlighted novel potential therapeutic targets. The aim of this review is to discuss the etiology, epidemiology, risk factors, clinical manifestations, recently disclosed pathogenic mechanisms underlying RP and their correlation with the available therapeutic options, focusing primarily on pRP and CTD-RP.
authors.affiliation: Internal Medicine, University of Bari Medical School, I-70124 Bari, Italy.
authors.forename: Marcella
authors.initials: M
authors.lastname: Prete
authors.affiliation: Internal Medicine, University of Bari Medical School, I-70124 Bari, Italy.
authors.forename: Maria Celeste
authors.initials: MC
authors.lastname: Fatone
authors.affiliation: Rheumatological and Autoimmune Systemic Diseases Units, University of Bari Medical School, I-70124 Bari, Italy.
authors.forename: Elvira
authors.initials: E
authors.lastname: Favoino
authors.affiliation: Rheumatological and Autoimmune Systemic Diseases Units, University of Bari Medical School, I-70124 Bari, Italy. Electronic address: federico.perosa@uniba.it.
authors.forename: Federico
authors.initials: F
authors.lastname: Perosa
mesh_terms: D000818:Animals; D003240:Connective Tissue Diseases / Q000209:etiology / Q000628:therapy; D003937:Diagnosis, Differential; D006801:Humans; D018384:Oxidative Stress; D011928:Raynaud Disease / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy*; D012307:Risk Factors
keywords: Autoantibodies; Connective tissue disease; Raynaud's phenomenon; Smooth muscle cells; Systemic sclerosis; Targeted therapy