Background Systemic sclerosis (SSc) is a chronic autoimmune inflammatory pathogenic disease of the connective tissue, characterized by progressive fibrosis thickening of skin and internal organs. SSc is caused by the accumulation of collagen (gastrointestinal system, heart, lung, kidney, joints and muscles) and widespread of vascular disease damage. The first vascular event is Raynaud’s phenomenon(RP).
Objectives In our study, we evaluated the efficacy of Aminaftone in the treatment of RP in 19 patients with SSc consecutively recruited and divided in two groups.
Methods Group A:9 Patients that not tolerated endothelin receptor antagonist (ERA) due to side effects (eg increased> 3 times the liver enzymes, headache, edema of lower limbs), in treatment with ACE inhibitors, prostanoids and Aminaftone.
Group B:10 Patients in treatment with ACE inhibitors, prostanoids and ERA All Patients were aged between 40 and 68 years (Group A 51.6±10.2;Group B 52.1±10.5), with SSc according to ACR criteria, presenting RP and DUs with an onset between 6 and 9 months.11 of the 19 patients had the limited form of SSc withpositive anticentromere antibody value and 8 of them had a diffuse form withpositive SCL-70 antibody value. The mid duration of SSc was 6.4 years with 10.8 years of average duration of onset of RP. The A group tooks three tablets of Aminaftone 75mg/day while the B group tooks two tablets of Bosentan 125mg/day. Follow-up was performed every 4 weeks for 6 months and each patient was given a diary to report on each check, which indicate:
- Date of onset of Raynaud’s Phenomenon
- Duration: minutes
- Raynaud’s Condition Score(RCS): Limitation of daily activity on a scale of 1 to 10 (meaning 10 as a total inability to do the activity)
- Pain VAS (1-10): 1 meaning the least pain and 10 as the maximum pain
- Number of daily attacks
- Ulcer onset (date)
- Location ulcer/s
Results The number of RP attacks in both groups of patients decreased from baseline to Week 24, obtaining statistically significance (ΔA-2,80 p-0,02;ΔB-3.10 p-0,01). The duration of the RP was decreased, but did not obtainstatistically significance (ΔA-10,90 ΔB-12,30). The RCS has shown a statistically significant improvement (ΔA-2,10 p-0,04;ΔB-2.3 p-0,03), as well as the pain VAS (ΔA-3,20 p-0,04;ΔB-3,5 p-0,02) at week 24. The number of DUs decreased in both group of patients and in most cases (especially in Group B), the lesions met complete resolution. Furthermore, in patients treated with Aminaftone, no extras DUs appeared. Our data showed a statistically significant improvement of baseline in the number of attacks of RP, VAS pain and RCS.
Conclusions In conclusion, the use of Aminaftone for the treatment of RP in SSc patients, seems to have been effective, with good tolerability and no adverse events. Although the ERA (Bosentan) is more effective, we believe that, at least for those patients whose therapy with ERA is not tolerated, Aminaftone may be a viable alternative treatment for RP. Long term studies with a wider coverage must be performed to assess the effects of Aminaftone.
References Kahaleh MB. Raynaud phenomenon and the vascular disease in scleroderma. Curr Opin Rheumatol 2004;16: 718–22.
Arefiev Kait et al. Endothelin Receptor Antagonists for the Treatment of Raynaud’s Phenomenon and Digital Ulcers in Systemic Sclerosis. International Journal of Rheumatology Volume 2011, Article ID 201787,7
Disclosure of Interest None Declared