Brief Report: Smoking in Systemic Sclerosis: A Longitudinal European Scleroderma Trials and Research Group Study

Jaeger, Veronika K.; Valentini, Gabriele; Hachulla, É.; Cozzi, Franco; Distler, Oliver; Airò, Paolo; Czirják, László; Allanore, Yannick; Siegert, Elise; Rosato, Edoardo; Matucci‐Cerinic, Marco; Caimmi, Cristian; Henes, Jörg; Carreira, Patrícia; Smith, Vanessa; Galdo, Francesco Del; Denton, Christopher P.; Ullman, S; Langhe, Ellen De; Riccieri, Valeria; Sancho, Juan José Alegre; Rednic, Simona; Müller‐Ladner, Ulf; Walker, Ulrich A.

doi:10.1002/art.40557

Cited by 17 publications

(12 citation statements)

References 16 publications

(22 reference statements)

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“…Interestingly, there were no significant differences in the prevalence of PH in patients with DU compared to those without DU history, differently from what we expected. In addition, smoking habit was associated with current DU, but not DU history, differently from what suggested by a previous systematic review [16] and a recent EUSTAR based prospective study [27].…”

Section: Discussioncontrasting

confidence: 88%

Use of vasoactive/vasodilating drugs for systemic sclerosis (SSc)-related digital ulcers (DUs) in expert tertiary centres: results from the analysis of the observational real-life DeSScipher study

et al. 2019

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Introduction: DeSScipher is the first European multicentre study on management of systemic sclerosis (SSc) and its Observational Trial 1 (OT1) evaluated the efficacy of different drugs for digital ulcer (DU) prevention and healing. The aim of this study was to assess current use of vasoactive/vasodilating agents for SSc related DU in the expert centres, by analysing the baseline data of the DeSScipher OT1 trial. Method: Baseline characteristics of patients enrolled in the OT1 and data regarding DU were analysed. Results: The most commonly used drugs, in both patients with and without DU, were calcium channel blockers (CCBs) (71.6%), followed by intravenous Iloprost (20.8%), endothelin receptor antagonists (ERAs) (20.4%) and phosphodiesterase 5 (PDE-5) inhibitors (16.5%). 32.6% of patients with DU and 12.8% patients without DU received two drugs (p<0.001), while 11.5% patients with DU and 1.9% without DU were treated with combination of three or more agents (p<0.001). Sixty-five percent of the patients with recurrent DU were treated with Bosentan and/or Sildenafil. However, 64 out of 277 patients with current DU (23.1%) and 101 (23.6%) patients with recurrent DU were on CCBs alone. Conclusions: Our study shows that CCBs are still the most commonly used agents for DU management in SSc. The proportion of patients on combination therapy was low, even in patients with recurrent DU: almost one out of four patients with current and recurrent DU was on CCBs alone. Prospective analysis is planned to investigate the efficacy of different drugs/drug combinations on DU healing and prevention.

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Section: Discussioncontrasting

confidence: 88%

Use of vasoactive/vasodilating drugs for systemic sclerosis (SSc)-related digital ulcers (DUs) in expert tertiary centres: results from the analysis of the observational real-life DeSScipher study

et al. 2019

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“…Patients should be counselled, and supported in their efforts, about the importance of smoking cessation because smoking promotes vasoconstriction. 93,94 Smoking has been reported to be associated with more severe digital vascular disease 93 including in relation to the intensity of smoking. 93,94 Patients should seek early medical advice about new and/or worsening ulcers, including potential signs of infection.…”

Section: General Approachmentioning

confidence: 99%

“…93,94 Smoking has been reported to be associated with more severe digital vascular disease 93 including in relation to the intensity of smoking. 93,94 Patients should seek early medical advice about new and/or worsening ulcers, including potential signs of infection. The development of persistent digital ischaemia should prompt the patient to seek emergency medical advice.…”

Section: General Approachmentioning

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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“…However, combining both predictors (S PO 2 and ARthritis) increased the prediction rate from 25.5% to 91.7% [80]. In another study, a combination of smoking history, age and D LCO % pred was used to predict risk of mortality [81] (interestingly, smoking alone has not been associated with a more rapid FVC decline in SSc) [82]. These studies suggest that composite measures and staging systems could be used in clinical practice to help discuss prognosis and guide clinical management.…”

Section: Composite Risk Scores In Ssc-ildmentioning

confidence: 99%

Natural variability in the disease course of SSc-ILD: implications for treatment

et al. 2021

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Interstitial lung disease (ILD) affects approximately 50% of patients with systemic sclerosis (SSc) and is the leading cause of death in SSc. Our objective was to gain insight into the progression of SSc-associated ILD (SSc-ILD). Using data from longitudinal clinical trials and observational studies, we assessed definitions and patterns of progression, risk factors for progression, and implications for treatment. SSc-ILD progression was commonly defined as exceeding specific thresholds of lung function worsening and/or increasing radiographic involvement. One definition used in several studies is decline in forced vital capacity (FVC) of ≥10%, or ≥5–10% plus a decline in diffusing capacity of the lung for carbon monoxide ≥15%. Based on these criteria, 20–30% of patients in observational cohorts develop progressive ILD, starting early in the disease course and progressing at a highly variable rate.Risk factors such as age, FVC, extent of fibrosis and presence of anti-topoisomerase I antibodies can help predict progression of SSc-ILD, though composite risk scores may offer greater predictive power. Whilst the variability of the disease course in SSc-ILD makes risk stratification of patients challenging, the decision to initiate, change or stop treatment should be based on a combination of the current disease state and the speed of progression.

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Brief Report: Smoking in Systemic Sclerosis: A Longitudinal European Scleroderma Trials and Research Group Study

Abstract: The known adverse effect of smoking on bronchial airways and alveoli is also observed in SSc patients; however, robust adverse effects of smoking on the progression of SSc-specific pulmonary or cutaneous manifestations were not observed.

Cited by 17 publications

References 16 publications

Use of vasoactive/vasodilating drugs for systemic sclerosis (SSc)-related digital ulcers (DUs) in expert tertiary centres: results from the analysis of the observational real-life DeSScipher study

Use of vasoactive/vasodilating drugs for systemic sclerosis (SSc)-related digital ulcers (DUs) in expert tertiary centres: results from the analysis of the observational real-life DeSScipher study

Raynaud phenomenon and digital ulcers in systemic sclerosis

Natural variability in the disease course of SSc-ILD: implications for treatment

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