Evolving Symptom Characteristics of Raynaud's Phenomenon in Systemic Sclerosis and Their Association With Physician and Patient‐Reported Assessments of Disease Severity

Pauling, John D; Reilly, Elizabeth; Smith, Theresa; Frech, Tracy

doi:10.1002/acr.23729

Cited by 27 publications

(18 citation statements)

References 17 publications

(26 reference statements)

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“…SSc patients can identify with distinct patterns of RP over time (that may reflect progression of vasculopathy) with established disease being associated with fewer 'stereotypical' attacks of RP, and more persistent features of tissue ischaemia. 18 Cold exposure is an important trigger for attacks of RP.…”

Section: Epidemiologymentioning

confidence: 99%

Raynaud phenomenon and digital ulcers in systemic sclerosis

et al. 2020

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Raynaud's phenomenon (RP) is a symptom complex related to impaired digital perfusion and can occur as a primary phenomenon or secondary to a wide range of underlying causes. RP occurs in virtually all patients with systemic sclerosis (SSc) and is often the earliest clinical manifestation in the natural history of the disease. Careful assessment is required in RP patients to avoid missing secondary causes of RP, including SSc. Digital ulcers (DUs) are a painful and disabling visible manifestation of the digital vascular injury. Significant progress has been made in the definition and assessment of DUs and understanding ulcer pathogenesis. There are a wide range of available treatments to both prevent and heal DUs; some of which are also used in RP management. The present review shall consider the assessment of patients with RP, including 'red flags' suggestive of SSc. We shall review the pathogenesis, definition and classification across the spectrum of SSc-DU disease, alongside a review on management approaches including drug therapies and surgery for SSc-RP and ulcers. We also highlight unmet needs and research priorities in SSc-RP and SSc-DUs and introduce the concept of a unified vascular phenotype in which vascular therapies may support disease modification strategies.

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Section: Epidemiologymentioning

confidence: 99%

Raynaud phenomenon and digital ulcers in systemic sclerosis

et al. 2020

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“…The relative importance of the various factors influencing a patient assessment of worsening symptoms is likely to vary significantly between patients, and this cannot be easily quantified in clinical studies. Also, there is the potential for underreporting the burden or change in disease status with increasing SSc duration as patients adapt to their disease and particular symptomatology (8). Of note, this study did not demonstrate a significant response shift across any of the domains of SSc evaluated, particularly when evaluating patient‐reported symptoms of RP and the development of digital ulcers.…”

Section: Discussionmentioning

confidence: 99%

“…Previous studies have demonstrated that patient assessment of the overall impact of RP on daily activities is associated with the presence of digital ulcers (6,7). Yet, studies that have evaluated the nature of patients’ RP have demonstrated a lack of association with RP pattern and clinical outcome, suggesting that there may be limits to the use of patient‐reported RP as a measure of disease activity (8). Potentially limiting the utility of patient‐assessed RP as a marker of disease activity is the marked response shift of RP symptoms associated with increasing SSc disease duration.…”

Section: Introductionmentioning

confidence: 99%

“…Potentially limiting the utility of patient‐assessed RP as a marker of disease activity is the marked response shift of RP symptoms associated with increasing SSc disease duration. The longer a patient has lived with RP, the better they are able to manage their symptoms, and therefore they generally report a less severe impact of RP symptoms (2,8).…”

Section: Introductionmentioning

confidence: 99%

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Can Patient‐Reported Symptoms Be Used to Measure Disease Activity in Systemic Sclerosis?

Ross

Stevens

Wilson

et al. 2020

Arthritis Care & Research

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Objective To evaluate the association between patient‐reported symptoms and changes in disease activity over time in systemic sclerosis (SSc). Methods Using data from 1,636 patients enrolled in the Australian Scleroderma Cohort Study, we used generalized estimating equations to determine the relationship between patient‐reported worsening of Raynaud’s phenomenon (RP), skin involvement, and breathlessness in the month preceding each study visit and features of disease activity in the corresponding organ systems. The associations between the following parameters were analyzed: patient‐reported worsening RP and the presence of new‐onset digital pitting and digital ulcers; patient‐reported worsening skin involvement and increasing modified Rodnan skin thickness score (MRSS); new areas of skin involvement and new‐onset joint contractures; patient‐reported worsening breathlessness and deteriorating respiratory functions test (RFT) results, indicated by a 10% decrease in forced vital capacity (FVC) and a 15% decrease in diffusing capacity for carbon monoxide (DLco), new‐onset interstitial lung disease (ILD), and new‐onset pulmonary arterial hypertension (PAH). Results We found a significant association between patient‐reported worsening RP and the presence of digital ulcers (odds ratio [OR] 1.53 [95% confidence interval (95% CI) 0.60–0.93]), patient‐reported worsening skin involvement and increasing MRSS (OR 2.10 [95% CI 1.54–2.86]), and worsening patient breathlessness and deteriorating RFTs (FVC OR 2.12 [95% CI 1.70–2.65]; DLco OR 1.97 [95% CI 1.34–2.02]), new‐onset ILD (OR 1.91 [95% CI 1.40–2.61]), and new‐onset PAH (OR 5.08 [95% CI 3.59–7.19]). Conclusion These results demonstrate that patient‐reported symptoms are associated with clinically meaningful changes in disease activity in patients with SSc. This suggests that when objective measures of change in disease status are unavailable, patient‐reported symptoms could be used to indicate a change in SSc disease activity.

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“…Ele é uma resposta vasoconstritora anormal das artérias e arteríolas digitais, em geral desencadeado pelo frio e provocando alteração da coloração das extremidades, principalmente das mãos e pés. O fenômeno gera palidez seguida de cianose e rubor (MAVERAKIS et al, 2013;PAULING et al, 2018).…”

Section: Introductionunclassified

Detecção e manejo de disfunções cardíacas em pacientes com esclerose sistêmica tratados com altas doses de ciclofosfamida seguidas por transplante de células-tronco hematopoéticas

Leopoldo¹

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DEDICATÓRIADedico esta tese aos meus pais, Francisco e Maria José, meu Porto seguro, exemplos de força e comprometimento, que tanto apoiaram e incentivaram o meu crescimento profissional, sempre acreditando nos meus sonhos. À eles, dedico todas as minhas vitórias, pois nunca mediram esforços para que eu chegasse até essa etapa de minha vida. Amo vocês. À Vitória ,linda da tia, que me fez conhecer o que é amor incondicional e a cada dia faz com que eu queira ser melhor, ser exemplo e estar presente. À minha família, pela compreensão e carinho ao longo do período de elaboração desta tese e desde sempre. AGRADECIMENTOSÀ Deus, sem o qual nada disso seria possível. À Profa. Dra. Renata Cristina de Campos Pereira Silveira, por me permitir ser sua aluna, pela sua orientação, apoio, por compartilhar toda sua sabedoria e ser exemplo na enfermagem. Obrigada por me acompanhar nesta jornada e por estimular meu interesse pelo conhecimento e possibilitar meu crescimento pessoal e profissional. Gratidão eterna! À Profa. Dra. Maria Carolina de Oliveira, sensibilidade, competência, inteligência, humana: um exemplo a ser seguido e admirado. Obrigda pela acolhida, paciência, incentivo e pelos conhecimentos compartilhados. Gratidão eterna!. . À Profa. Dra. Denise de Andrade, agradeço por ter acreditado naquela estudante universitária mas apaixonada pela pesquisa. Obrigada por me introduzir nesse universo maravilhoso durante a iniciação científica e compartilhar todo seu conhecimento. Minha admiração e gratidão sempre! À Dra Juliana Elias um exemplo de profissional , competente, sábia mas sobretudo humana. Sua ajuda foi fundamental nesse projeto. À Paty, Tia Ezildinha, e toda minha família pelo apoio mesmo a distância, sempre tão perto. Ao Luis Paulo (Kinho) pela ajuda com os dados. Ao Luciano, mais que um amigo, um irmão que a vida me deu. A toda Equipe interdisciplinar da Unidade de Terapia Imunológica representada pela enfermeira Andréia. Obrigada pela colaboração na coleta das amostras, pelos "cafés", convivência diária e colaboração na escala. Aos enfermeiros e a minha equipe do Hospital Santa Tereza, representados pela enfermeira Dgmar. Obrigada por entender e me ajudar a realizar esse projeto pessoal A Rubens, Muriel, Basílica e Juliana do laboratório de Imunomolecular pela paciência e disponibilidade em compartilharem seus conhecimentos A Júlia do Hemocentro pela disponibilidade de compartilhar seu conhecimento e processar as amostras de sangue A Marília por toda ajuda e disponibilidade em auxiliar no processamento das amostras Aos pacientes pelo sentimento de amor ao próximo e doação Aos meus pais, modelos de coragem e luta, por todo apoio e amor incondicional, incentivo, paciência e total ajuda na superação dos obstáculos que ao longo dessa caminhada foram surgindo. "As pessoas mais bonitas que conhecemos São aquelas que conheceram o sofrimento, Conheceram a derrota, Conheceram o esforço, Conheceram a perda E encontraram em seu caminho para fora das profundezas Essas pessoas têm uma apreciação, uma sensibilidade e uma compr...

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Evolving Symptom Characteristics of Raynaud's Phenomenon in Systemic Sclerosis and Their Association With Physician and Patient‐Reported Assessments of Disease Severity

Cited by 27 publications

References 17 publications

Raynaud phenomenon and digital ulcers in systemic sclerosis

Raynaud phenomenon and digital ulcers in systemic sclerosis

Can Patient‐Reported Symptoms Be Used to Measure Disease Activity in Systemic Sclerosis?

Detecção e manejo de disfunções cardíacas em pacientes com esclerose sistêmica tratados com altas doses de ciclofosfamida seguidas por transplante de células-tronco hematopoéticas

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