Raynaud's phenomenon

Publication date: Available online 29 January 2020Source: Best Practice &Research Clinical RheumatologyAuthor(s): Ariane L. Herrick, Fredrick M. WigleyAbstractRaynaud's phenomenon (RP) is common, affecting approximately 5% of the population, and is important to the rheumatologist because it is often the presenting symptom of connective tissue disease, especially of systemic sclerosis (SSc)-spectrum disorders. RP therefore provides a window of opportunity for early diagnosis. When RP is associated with SSc it is particularly challenging to treat.This review begins with a discussion of some of the recent advances in our understanding of the pathogenesis of RP: it is through increased understanding of the complex pathophysiology of RP that we are most likely to develop new therapies. The following questions are then addressed (with three clinical scenarios demonstrating key principles of assessment and management):1. How can we predict underlying connective tissue disease in the patient presenting with Raynaud's?2. How can we measure severity of Raynaud's?3. What are the latest advances in treatment of connective tissue disease-related digital vasculopathy?
Source: Best Practice and Research Clinical Rheumatology - Category: Rheumatology Source Type: research

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CONCLUSION: This study showed that patients with very early SSc are a mixture of patients with mild or early disease. This needs to be considered in clinical practice for risk stratification and for the study design of patients considered as early SSc. PMID: 32173655 [PubMed - as supplied by publisher]
Source: Journal of Rheumatology - Category: Rheumatology Tags: J Rheumatol Source Type: research
ConclusionOverall, RP and SSc internet-based information is of low quality and inadequate readability. The RP and SSc international community should strongly consider developing an information standard for internet-based resources for healthcare users.Key points:• Patients with SSc and RP are increasingly using internet/online sources of information and support.• RP represents an important opportunity for the early diagnosis of SSc.• The overall quality and readability of internet-based RP and SSc information is poor.• Internet-based RP and SSc information requires improvement to facilitate early diagno...
Source: Clinical Rheumatology - Category: Rheumatology Source Type: research
Conclusions Patients with SSc may present with a clinically and pathologically distinct myopathy. A prompt and correct diagnosis has important implications for treatment. Finally, TSP-1 may participate in the pathologic changes observed in muscle.
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: MRI, All Neuromuscular Disease, Muscle disease Article Source Type: research
Authors: Rezus E, Burlui AM, Gafton B, Stratulat TA, Zota GR, Cardoneanu A, Rezus C Abstract Systemic sclerosis (SSc) is a rare and complex autoimmune disease associated with poor vital and functional outcomes. The functional hindrance in patients derives from various disease-specific manifestations, including Raynaud's phenomenon and digital ulcers (DUs). Bosentan is an endothelin receptor antagonist capable of preventing the appearance of new DUs in patients with scleroderma. The present study aimed to evaluate the effects of Bosentan on the severity of Raynaud's phenomenon, DU-related symptoms and functional imp...
Source: Experimental and Therapeutic Medicine - Category: General Medicine Tags: Exp Ther Med Source Type: research
Nature Reviews Rheumatology, Published online: 25 February 2020; doi:10.1038/s41584-020-0386-4Raynaud phenomenon and digital ulcers are some of the most common and difficult to treat manifestations of systemic sclerosis. In this Review, the authors outline how to assess and treat these conditions, and also discuss unmet clinical needs.
Source: Nature Reviews Rheumatology - Category: Rheumatology Authors: Source Type: research
This study examined the relationship between global longitudinal strain (GLS) and pulmonary function tests (PFT) in patients with systemic sclerosis (SS) and normal ejection fraction (EF) and pulmonary artery pressure (PAP) and healthy controls. Sixty patients in two groups underwent extensive screening, including echocardiography, physical examination, the modified Rodnan Skin Score, and pulmonary function tests. Pulmonary interstitial disease was diagnosed by the pulmonary function test and by CT scan in case of indication. GLS score was computed as the mean peak systolic strain for 17 segments. The mean GLS score was &m...
Source: The International Journal of Cardiovascular Imaging - Category: Radiology Source Type: research
CONCLUSION: Patients consistently reported a positive effect of treatment with prostacyclin derivatives on Raynaud's phenomenon, acral ulcerations, pain and daily restrictions and felt well and safely cared for during inpatient treatment. These positive effects in the patients' perceptions provide crucial information supporting and confirming the current European and international treatment recommendations. PMID: 32040755 [PubMed - as supplied by publisher]
Source: Zeitschrift fur Rheumatologie - Category: Rheumatology Authors: Tags: Z Rheumatol Source Type: research
Source: Clinical Rheumatology - Category: Rheumatology Source Type: research
CONCLUSIONS: Dilatation score performs best of all semi-quantitative NVC parameters in diagnosing SSc. In addition, our study confirms earlier reports that worse capillaroscopy pattern at baseline correlates with higher likelihood for adverse prognosis. PMID: 31969216 [PubMed - as supplied by publisher]
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
Publication date: Available online 10 January 2020Source: Autoimmunity ReviewsAuthor(s): Vanessa Smith, Ariane L. Herrick, Francesca Ingegnoli, Nemanja Damjanov, Angelis, Christopher P. Denton, Oliver Distler, Karinna Espejo, Ivan Foeldvari, Tracy Frech, Boris Garro, Marwin Gutierrez, Genevieve Gyger, Eric Hachulla, Roger Hesselstrand, Annamaria Iagnocco, Cristiane Kayser, Karin Melsens, Ulf Müller-Ladner, Carmen PizzorniAbstractCapillaroscopy is a non-invasive and safe tool which allows the evaluation of the morphology of the microcirculation. Since its recent incorporation in the 2013 American College of Rheumatolog...
Source: Autoimmunity Reviews - Category: Allergy & Immunology Source Type: research
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