scholarly journals Endothelin Receptor Antagonists for the Treatment of Raynaud's Phenomenon and Digital Ulcers in Systemic Sclerosis

2011 ◽  
Vol 2011 ◽  
pp. 1-7 ◽  
Author(s):  
Kait Arefiev ◽  
David F. Fiorentino ◽  
Lorinda Chung
Keyword(s):  
Systemic Sclerosis ◽  
Case Reports ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Digital Ulcers ◽  
Endothelin Receptor Antagonists ◽  
Endothelin Receptor ◽  
Significant Morbidity ◽  
Receptor Antagonists ◽  
Internal Organs

Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin, internal organs, and widespread vasculopathy. Raynaud's phenomenon and digital ulcers are vascular manifestations of this disease and cause significant morbidity. Current treatments are only moderately effective in reducing the severity of Raynaud's in a portion of patients and typically do not lead to substantial benefit in terms of the healing or prevention of digital ulcers. Several studies have evaluated the efficacy of targeting the vasoconstrictor endothelin-1 for the treatment of systemic sclerosis-associated vascular disease. The purpose of this paper is to summarize the published studies and case reports evaluating the efficacy of endothelin receptor antagonists in the treatment of Raynaud's phenomenon and digital ulcers associated with systemic sclerosis.

Iloprost infusion through elastomeric pump in the treatment of Raynaud’s phenomenon and digital ulcers

2018 ◽  
Vol 4 (1) ◽  
pp. NP1-NP4
Author(s):  
Maria Helena Fartura Braga Temido ◽  
Manuel Gomes ◽  
Francisco Parente ◽  
Lèlita Santos
Keyword(s):  
Systemic Sclerosis ◽  
Case Reports ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Vascular Involvement ◽  
Systemic Autoimmune Disease ◽  
Portable Devices ◽  
Digital Ulcers ◽  
Critical Ischaemia ◽  
Iloprost Infusion

Introduction: Systemic sclerosis is a systemic autoimmune disease characterized by microangiopathy and fibroblast dysfunction resulting in fibrosis of the skin and internal organs. Raynaud’s phenomenon and digital ulcers are the main clinical features of vascular involvement. Treatment with iloprost is recommended to reduce the frequency and severity of Raynaud’s phenomenon attacks and to heal active digital ulcer. Classical forms of treatment require admission to a ward or day-hospital unit to ensure safety during infusion, causing disruption of the patient’s normal daily life and resulting in significant costs with hospitalization. Nowadays, new portable devices, of which the elastomeric pump is an example, are becoming available in order to avoid hospitalization. Case reports: We describe five cases of patients with systemic sclerosis or mixed connective tissue disease and severe Raynaud’s phenomenon/critical ischaemia or active digital ulcers that were successfully treated with iloprost infusion through elastomeric pump without experiencing any side effects. We present our unit’s protocol for ambulatory infusion. Discussion/conclusion: Our case reports and a brief review of literature prove that iloprost infusion through elastomeric pump is safe, easy and well tolerated and might even improve patient compliance with treatment. Meanwhile, it may also decrease the economic burden of hospitalization with these patients.

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.

Possible benefit of tadalafil cream for the treatment of Raynaud’s phenomenon and digital ulcers in systemic sclerosis

2020 ◽  
Vol 39 (3) ◽  
pp. 963-965
Author(s):  
Andreu Fernández-Codina ◽  
Mikameh Kazem ◽  
Janet E. Pope
Keyword(s):  
Systemic Sclerosis ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Digital Ulcers

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.

Endothelin Receptor Antagonists as Disease Modifiers in Systemic Sclerosis

2011 ◽  
Vol 10 (1) ◽  
pp. 19-26 ◽  
Author(s):  
Nagalakshmi Shetty ◽  
Chris T. Derk
Keyword(s):  
Systemic Sclerosis ◽  
Endothelin Receptor Antagonists ◽  
Endothelin Receptor ◽  
Receptor Antagonists
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