Raynaud’s Phenomenon and Plasma Endothelin: Correlations with Capillaroscopic Patterns in Systemic Sclerosis

2009 ◽  
Vol 36 (6) ◽  
pp. 1235-1239 ◽  
Author(s):  
ALBERTO SULLI ◽  
STEFANO SOLDANO ◽  
CARMEN PIZZORNI ◽  
PAOLA MONTAGNA ◽  
MARIA ELENA SECCHI ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Plasma Levels ◽  
Rating Scale ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Digital Ulcers ◽  
Microvascular Damage ◽  
Morphological Alterations ◽  
Primary Raynaud’S Phenomenon ◽  
Clinical Measures

Objective.We evaluated endothelin (ET)-1 plasma levels and some clinical measures in patients with primary Raynaud’s phenomenon (PRP), and in patients with systemic sclerosis (SSc) and secondary RP (SRP), in the latter according to their different nailfold videocapillaroscopy (NVC) patterns of microangiopathy (early, active, and late).Methods.Ninety-nine patients with SSc, 49 with PRP, and 45 control subjects were studied. NVC was performed in all patients to distinguish the pattern of microvascular damage, and the morphological alterations were scored by a semiquantitative rating scale. ET-1 plasma levels were evaluated in all individuals by ELISA.Results.ET-1 plasma levels were significantly higher (p = 0.001) in patients with both PRP and SRP, compared to controls. A significant positive correlation (p = 0.03) was found between ET-1 plasma levels and SRP duration, but not between ET-1 plasma levels and PRP duration. Significant correlations were observed in patients with SSc between ET-1 plasma levels and clinical measures (e.g., digital ulcers), as well as the score value of single NVC measures, such as the number of capillaries, “ramified” capillaries, and enlarged capillaries (p < 0.05). Finally, the highest ET-1 plasma levels were found in patients with SSc showing the late pattern of microangiopathy when compared to the early pattern (p = 0.03) and to controls (p = 0.003).Conclusion.Highest ET-1 plasma levels were detected in the more advanced stage of the SSc microangiopathy, namely the late NVC pattern, characterized by capillary loss and increased tissue fibrosis; this might support the involvement of ET-1 in the progression of the microvascular/fibrotic SSc damage.

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 INCREASED NAILFOLD CAPILLARY DIMENSIONS IN PRIMARY RAYNAUD'S PHENOMENON AND SYSTEMIC SCLEROSIS

Rheumatology ◽  
1995 ◽  
Vol 35 (11) ◽  
pp. 1127-1131 ◽  
Author(s):  
M. BUKHARI ◽  
A. L. HERRICK ◽  
T. MOORE ◽  
J. MANNING ◽  
M. I. V. JAYSON
Keyword(s):  
Systemic Sclerosis ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Primary Raynaud’S Phenomenon

Characterization of Endothelin-Binding Sites in Human Skin and their Regulation in Primary Raynaud's Phenomenon and Systemic Sclerosis

1993 ◽  
Vol 101 (1) ◽  
pp. 73-78 ◽  
Author(s):  
Gregory A Knock ◽  
Giorgio Terenghi ◽  
Christopher B Bunker ◽  
Helen A. Bull ◽  
Pauline M. Dowd ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Human Skin ◽  
Binding Sites ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Primary Raynaud’S Phenomenon
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