Efficacy of bosentan in the treatment of Raynaud’s phenomenon in patients with systemic sclerosis never treated with prostanoids

Parisi, Simone; Bruzzone, Maria; Vittorio, Chiara Centanaro Di; Laganà, Angela; Peroni, Clara Lisa; Fusaro, Enrico

doi:10.4081/reumatismo.2013.691

Cited by 6 publications

(6 citation statements)

References 15 publications

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“…It is too underpowered to detect small treatment effect. Nevertheless as previously shown by other authors in treatment with ERA and PHE-5 Inhibitors, our data highlighted a statistically significant improvement of RP [12,21,27,28], while another studies failed to document any beneficial effect on VAS, RP or development of digital ulcers [29,30,31]. These phenomena determine patient worsening of the quality of life, not only in terms of pain, but also due to the extreme difficulty in performing normal daily activities.…”

Section: Discussionsupporting

confidence: 78%

“…Same results have been highlighted by other studies where endothelin receptor antagonists were used [21,22,23]. Aminaftone but these are studies on a small sample of patients [20], while there are some studies (but non-blinded approach) of bosentan in the treatment of Raynaud's Phenomenon and digital ulcers in SSc patients [15,25,26,27]. Thus, this preliminary study it's restricted by its limitations.…”

Section: Discussionmentioning

confidence: 52%

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Aminaftone in the Treatment of Raynaud’s Phenomenon in Systemic Sclerosis: New Perspectives

Parisi¹

2015

AJIM

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Objective: The aim of this study was to assess the efficacy (in terms of improved VAS Pain, Raynaud's Phenomenon attacks frequency and Raynaud's Condition Score) of the Aminaftone in patients with Raynaud's Phenomenon (RP) secondary to Systemic Sclerosis (SSc). Methods: Open label controlled study. We evaluated the efficacy of Aminaftone in the treatment of secondary RP in 108 patients with SSc consecutively recruited and divided in two groups. Group A: 51 Patients in treatment with Calcium Channel Blockers, prostanoids (iloprost 2ng [min/kg every 4 weeks), Endothelin Receptor Antagonist and Phosphodiesterase-5 inhibitors (Standard of Care). Group B: 57 Patients in treatment with Calcium Channel Blockers, Prostanoids, Endothelin Receptor Antagonist and Phosphodiesterase-5 inhibitors and Aminaftone (Standard of care + Aminaftone). Results: The number of RP attacks in group treated with Aminaftone (Group B) was lower than the group treated with standard therapy (Group A) from baseline to Week 48, obtaining statistically significance (Δ A-1.50 Δ B-2.10 p=0.02 95%CI). The RCS was statistically significantly different (Δ A-1.60 Δ B-2.50 p=0,04 95% CI), as was the pain VAS(Δ A-20.80 Δ B-30.60 p=0.04 95%CI) at week 48. Conclusion: The use of Aminaftone was well tolerated and improved RP as measured by RCS and pain when added to standard of care. A well-designed randomized controlled trial is needed to determine if these preliminary findings are supported.

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Section: Discussionsupporting

confidence: 78%

Section: Discussionmentioning

confidence: 52%

Aminaftone in the Treatment of Raynaud’s Phenomenon in Systemic Sclerosis: New Perspectives

Parisi¹

2015

AJIM

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“…W badaniu RAPIDS-2 wykazano, że istotnie zmniejsza on częstość występowania nowych owrzodzeń palców u pacjentów z twardziną [23]. Bozentan może być stosowany jako lek alternatywny u pacjentów z przeciwwskazaniami do leczenia prostanoidami [26].…”

Section: Raynaudaunclassified

“…In the RAPIDS-2 trial, bosentan was found to significantly decrease the incidence of new digital ulcers in patients with scleroderma [23]. It can be an alternative for patients with contraindications to prostanoids [26].…”

Section: Raynaudamentioning

confidence: 99%

Raynaud’s phenomenon as an interdisciplinary problem

Gorska¹,

Szczerkowska-Dobosz²,

Purzycka-Bohdan³

et al. 2017

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Raynaud's phenomenon is one of the most common disorders of the blood vessels, which usually affects fingers and toes. Raynaud's disease, also called primary Raynaud's phenomenon, is diagnosed when the cause is unknown, whereas Raynaud's syndrome or secondary Raynaud's phenomenon occurs as a skin manifestation of concomitant disease. The most common trigger is exposure to cold. Specific clinical features allow a quick diagnosis. Raynaud's phenomenon usually does not require pharmacologic treatment, but in some cases symptoms are severe and pharmacotherapy is necessary. Calcium channel blockers are usually used as a first-line treatment. Alternative forms of therapy include angiotensin-converting-enzyme inhibitors, α1-adrenergic blockers, phosphodiesterase-5 inhibitors, endothelin antagonists, nitrates, antioxidants, statins, botulinum toxin, and even autologous fat grafting. StReSzczeNieObjaw Raynauda jest jedną z najczęstszych reakcji skurczowych naczyń krwionośnych, zazwyczaj dotyczy palców rąk i stóp. Choroba Raynauda, inaczej zwana pierwotnym objawem Raynauda, jest rozpoznawana, gdy przyczyna schorzenia jest nieznana. Zespół Raynauda lub wtórny objaw Raynauda współtowarzyszy innym chorobom. Najczęstszym czynnikiem prowokującym skurcz naczyń jest ekspozycja na zimno. Charakterystyczny obraz kliniczny zwykle pozwala na szybkie ustalenia rozpoznania. Objaw Raynauda u większości chorych nie wymaga leczenia farmakologicznego, ale u niektórych osób objawy są nasilone i farmakoterapia staje się niezbędna. Leki z grupy antagonistów wapnia są często stosowane w leczeniu pierwszego rzutu. Alternatywne formy terapii obejmują: inhibitory konwertazy angiotensyny, leki α1-adrenergiczne, inhibitory fosfodiesterazy 5, antagonistów endoteliny, azotany, przeciwutleniacze, statyny, toksynę botulinową, a nawet autologiczne przeszczepy tkanki tłuszczowej.

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“…The involvement of ET-1 in the elicitation of pain in peripheral locations has been well documented by administration of exogenous ET-1 in both humans and animal models of nociception 67-71 . Moreover, several studies have demonstrated the ability of ET receptor antagonists to alleviate clinical pain in patients with systemic sclerosis and Raynaud's phenomenon, two conditions in which disease manifestations share some similarity with SCD 72-74 . Nevertheless, two other studies have demonstrated negative results in these conditions 75,76 .…”

Section: Introductionmentioning

confidence: 99%

Endothelin receptor antagonists in sickle cell disease: A promising new therapeutic approach

Fox

Kasztan

2016

Life Sciences

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Sickle cell disease (SCD) is a genetic hematologic disorder that is characterized by a variety of potentially life threatening acute and chronic complications. Currently, hydroxyurea is the only clinically approved pharmacological therapy for the treatment of SCD, and the continued prevalence of severe disease complications underscores the desperate need for the development of new therapeutic agents. Central features of the sickle cell disease milieu, including hypoxia, oxidative stress, and thrombosis, are established enhancers of endothelin-1 (ET-1) synthesis. This conceptual connection between ET-1 and SCD was confirmed by multiple studies that demonstrated markedly elevated plasma and urinary levels of ET-1 in SCD patients. Direct evidence for the involvement of ET-1 signaling in the development of SCD pathologies has come from studies using endothelin receptor antagonists in SCD mice. This review summarizes recent studies that have implicated ET-1 signaling as a mechanistic contributor to renal, vascular, pulmonary, and nociceptive complications of sickle cell disease and discusses the potential for the use of ET receptor antagonists in the treatment of SCD.

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Efficacy of bosentan in the treatment of Raynaud’s phenomenon in patients with systemic sclerosis never treated with prostanoids

Cited by 6 publications

References 15 publications

Aminaftone in the Treatment of Raynaud’s Phenomenon in Systemic Sclerosis: New Perspectives

Aminaftone in the Treatment of Raynaud’s Phenomenon in Systemic Sclerosis: New Perspectives

Raynaud’s phenomenon as an interdisciplinary problem

Endothelin receptor antagonists in sickle cell disease: A promising new therapeutic approach

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