Background Raynaud Phenomenon (RP) is present in more than 90% of the patients with Systemic sclerosis (SSc) and it may progress to scars, ulcers, necrosis resulting in an incapacitating problem for many of them.
Objectives To evaluate the efficacy of the most common drugs used in clinical practice in SSc patients with RP (SSc-RP).
Methods A systematic literature search was performed in Medline, EMBASE and Cochrane Central Register of Controlled Trials (from the beginning to October 2011) and a manual search. We selected meta-analysis, systemic literature reviews, randomized clinical trials (RCT) and high quality cohorts studies (English, Spanish) which included SSc-RP (limited cutaneous and diffuse) patients, aged 18 years or above, with RP and/or digital ulcers on: calcium channel blockers, ACEI,ARB, endothelin receptor antagonist, phosphodiesterase inhibitors, prostanoids, antioxydants, NAC, SRA, cyclofenil, atorvastatin, stanazolol, hyperbaric oxygen, ulcers topical treatment, electrical neurostimulator, sympatectomy or nitroglycerine. Efficacy was evaluated as: number of RP episodes, RP severity, time without flares, ulcers improvement and healing, change of ulcers size, number of new ulcers, etc. Studies including patients with primary RF and basic science were excluded. Title and abstract selection and subsequent detailed review of selected articles were independently performed by two reviewers, BN and CP. Evidence tables were generated. The included studies quality was graded using the 2001 Oxford Levels of Evidence Scale, and results expressed as level of evidence (LE), recommendation grade (RG).
Results A total of 1,541 studies were identified, of which 29 fulfilled inclusion criteria. Quality was good in 19/29 (65.5%) studies. We found a great variability in the outcomes evaluated in the included studies. The efficacy in terms of level of evidence and grade of recommendation in SSc-RP patients are as follows: alpha adrenergic blockers: prazosin (LE 1a, GR A), OPC-2826 (LE 1c, GR B); ACEI: quinapril (LE 1a, GR A), enalapril (LE 1c, GR B); prostanoids: Iloprost iv (LE 1a, GR A), beroprost (LE 1a, GR A), cisoprost (LE 1b, GR B), misoprostol (LE 1c, GR B); endothelin receptor antagonist: bosentan (LE 1a, GR A); phosphodiesterase inhibitors: tadalafil (LE 1a, GR A), sildenafil (LE 1c, GR B); topic treatments: MQQx-503 (LE 1a, GR A), DMSO (LE 1b, GR B); other drugs: cyclofenil (LE 1b, GR B), atorvastatin (LE 1b, GR B), antioxydants (LE 1c, GR B), stanozolol (LE 1c, GR B).
Conclusions The evidence on the efficacy of the different pharmacological treatments for SSc-RP patients is heterogeneous. The best evidence regarding to the efficacy in SSc-RP patients was found in the following drugs: calcium channel blockers, ACEI, prostanoids, tadalafil and bosentan. There is a lack of high quality prospective studies.
Disclosure of Interest None Declared