scholarly journals Combination therapy with bosentan and sildenafil for refractory digital ulcers and Raynaud’s phenomenon in a 30-year-old woman with systemic sclerosis: Case report and literature review

2019 ◽  
Vol 5 (2) ◽  
pp. 159-164 ◽  
Author(s):  
Jan-Gerd Rademacher ◽  
Chris Wincup ◽  
Björn Tampe ◽  
Peter Korsten
Keyword(s):  
Case Report ◽  
Systemic Sclerosis ◽  
Literature Review ◽  
Combination Therapy ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Digital Ulcers ◽  
Channel Blockers ◽  
Tissue Necrosis ◽  
Diffuse Systemic Sclerosis

Background: Systemic sclerosis is a rare autoimmune disease characterized by skin and organ fibrosis, and vasculopathy. Raynaud’s phenomenon is almost universally present in systemic sclerosis and can be the most debilitating symptom. Raynaud’s phenomenon may lead to the development of digital ulcers, potentially complicated by infection, tissue necrosis, and auto-amputation. Recommended treatments have variable efficacy. Methods: We report the case of a 30-year-old woman with diffuse systemic sclerosis suffering from severe Raynaud’s phenomenon and digital ulcers with digital tissue necrosis who was treated with combination therapy of an endothelin receptor antagonist and phosphodiesterase 5 inhibitor. In addition, we reviewed the literature on the topic. Results: Previous therapy with calcium-channel blockers, intravenous iloprost, and bosentan had all failed to control symptoms. We added sildenafil in combination with bosentan and observed a rapid and sustained treatment effect. Raynaud’s phenomenon severity, number of attacks, and attack duration decreased within 2 weeks of initiating treatment. Furthermore, this resulted in the healing of established digital ulcers. Conclusion: Our case report suggests that combination therapy may be a feasible treatment for the most severely affected and refractory patients. In our literature review, we found one retrospective study and three additional cases with similarly encouraging results.

Management of Raynaud’s phenomenon in systemic sclerosis—a practical approach

2019 ◽  
Vol 4 (2) ◽  
pp. 102-110 ◽  
Author(s):  
Andreu Fernández-Codina ◽  
Esperanza Cañas-Ruano ◽  
Janet E Pope
Keyword(s):  
Systemic Sclerosis ◽  
Raynaud’S Phenomenon ◽  
Treatment Options ◽  
Raynaud's Phenomenon ◽  
Practical Approach ◽  
Digital Ulcers ◽  
Channel Blockers ◽  
Tissue Ischemia ◽  
Multiple Variables ◽  
Third Line

Raynaud’s phenomenon is nearly universal in systemic sclerosis. Vasculopathy is part of systemic sclerosis. Raynaud’s phenomenon can cause of complications and impairment, especially when tissue ischemia and digital ulcers develop. There are many treatment options for Raynaud’s phenomenon in systemic sclerosis often with sparse data and few robust studies comparing the different treatment options. Recommendations from guidelines usually include calcium channel blockers as first-line pharmacological treatment. In the clinical setting, multiple variables such as financial factors, geography where access to medications varies, and patient factors, baseline hypotension, can influence the treatment for Raynaud’s phenomenon and digital ulcers. Prostacyclins and PDE-5 inhibitors are reserved for more severe Raynaud’s phenomenon or healing of digital ulcers. Prevention of digital ulcers may also include endothelin receptor blocker (bosentan) in some countries. Other treatments had less consensus. Algorithms developed by systemic sclerosis experts might be helpful in deciding which treatment to choose for each setting, using a step-wise strategy, which intends to complement guidelines. This review focuses on a practical approach to the treatment of Raynaud’s phenomenon and digital ulcers in systemic sclerosis, based on algorithms designed by systemic sclerosis experts using consensus, and we review the evidence that supports treatment from initial to second and third-line options.

scholarly journals Treatment of digital ulcers with oxygen-ozone therapy in a patient with systemic sclerosis

Ozone Therapy ◽  
2017 ◽  
Vol 2 (2) ◽  
Author(s):  
Hilary Riva ◽  
Brenno Balestra ◽  
Olivier Giannini ◽  
Giuseppe Bonforte
Keyword(s):  
Systemic Sclerosis ◽  
Mechanism Of Action ◽  
Raynaud’S Phenomenon ◽  
Oxygen Metabolism ◽  
Raynaud's Phenomenon ◽  
Gas Mixture ◽  
Digital Ulcers ◽  
Ozone Therapy ◽  
Therapeutic Program

Patients with systemic sclerosis often suffer from Raynaud’s phenomenon and in the most severe cases it causes digital ulceration and difficult healing. Treatment for these complications are not always resolutive and sometimes not well tolerated. The O2O3 therapy is a gas mixture that has several demonstrated effects including antiseptic, painkiller, microcirculation vasodilator, oxygen metabolism regulator and immunomodulation, and it can be personalised, administered to individual patient requirements. We decided to offer this treatment to our patients after the failure of traditional drugs and surgery without influencing the therapeutic program. The results obtained are interesting, and though more studies are needed to better understand the exact mechanism of action of O2O3 therapy, we believe that in the meantime, this therapy should be considered as treatment for the most severe cases.

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