Consensus best practice pathway of the UK Systemic Sclerosis Study group: management of cardiac disease in systemic sclerosis

Bissell, Lesley-Anne; Anderson, Marina; Burgess, Malcolm; Chakravarty, Kuntal; Coghlan, Gerry; Dumitru, Raluca B; Graham, Lee N.; Ong, Voon H; Pauling, John D; Plein, Sven; Schlosshan, Dominik; Woolfson, Peter

doi:10.1093/rheumatology/kew488

Cited by 87 publications

(72 citation statements)

References 41 publications

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“…Применение ГК в дозах >15 мг/сут нежелательно из-за риска развития склеродермического почечного криза. Кроме того, используется ЦФ, однако применять его следует с осторожностью -препарат может оказывать кардиотоксический эффект в больших дозах [21]. Также применяются ММФ и метотрексат.…”

Section: Discussionunclassified

“…Но следует отметить, что на фоне приема ММФ отмечены единичные случаи экссудативного и адгезивного перикардита [22]. Как возможные неблагоприятные реакции при лечении метотрексатом были зафиксированы случаи перикардита, перикардиального выпота, гипотензия и тромбоэмболические события [21]. Но по данным рандомизированных контролируемых исследований метотрексата у пациентов с ССД какой-либо кардиотоксичности выявлено не было [23].…”

Section: Discussionunclassified

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Effect of rituximab on heart involvement in systemic sclerosis

Garzanova¹,

Ананьева²,

Koneva³

et al. 2019

Naučno-praktičeskaâ revmatologiâ

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As of now, there has been a great body of data on the use of rituximab (RTM) in systemic sclerosis (SS), mainly on its positive effect on skin fibrosis and lung injury. However, information is still scarce about the effect of RTM on other organs and systems, namely the heart affected by SS.Objective: to assess the time course of changes in the signs of heart involvement in SS patients one year after initiation of RTM therapy.Subjects and methods. The paper gives data on changes in cardiac disorders in 71 patients with SS one year after the prescription of RTM.Results and discussion. The rate of cardiac rhythm and conduction disorders and diastolic dysfunction was unchanged. At the same time, a significant increase in left ventricular ejection fraction (EF) and a reduction in the severity of dyspnea was achieved, which correlated with improved lung function (a significant rise in forced vital capacity). The results of treatment in three patients with predominant heart involvement associated with SS (coronary heart disease and hypertension were ruled out) were considered in detail. These patients displayed pronounced positive changes as increased EF, less severe cardiac arrhythmias, reduced chronic heart failure, better quality of life, as well as the synchronicity of lower disease activity, less skin induction, improved lung function, and stabilized pulmonary artery systolic pressure.Conclusion. RTM in combination with traditional therapy can be considered as a potentially effective drug for the treatment of heart involvement in SS.

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Section: Discussionunclassified

Effect of rituximab on heart involvement in systemic sclerosis

Garzanova¹,

Ананьева²,

Koneva³

et al. 2019

Naučno-praktičeskaâ revmatologiâ

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“…• The remaining 19 (38%) patients experienced no such symptoms/events during that time (the "no-event group"). All patients had undergone standardized echocardiographic evaluation [12] within one week of recruitment without identification of any abnormalities and all were evaluated using a 1.5T MR system.…”

Section: Patientsmentioning

confidence: 99%

“…The first non-invasive imaging modality usually employed for the cardiovascular assessment of SSc patients is echocardiography, which has the advantage of being inexpensive, fast and widely available [12]. However, it suffers from a number of drawbacks including but not limited to dependence of image quality on sufficient visual window and an inability to characterise myocardial tissues as to the presence of oedema and fibrosis [13].…”

Section: Introductionmentioning

confidence: 99%

Cardiovascular Magnetic Resonance Identifies High-Risk Systemic Sclerosis Patients with Normal Echocardiograms and Provides Incremental Prognostic Value

et al. 2019

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Background: Acute cardiac events are a significant contributor to mortality in systemic sclerosis (SSc). However, echocardiographic evaluation may be deceptively normal during an acute presentation. We hypothesized that in diffuse SSc patients presenting with acute cardiac events and a normal echocardiogram, cardiovascular magnetic resonance (CMR) would have incremental diagnostic/prognostic value. Methods. 50 consecutive diffuse SSc patients with normal echocardiograms were evaluated using a 1.5T system. A total of 27 (63%) had experienced an acute cardiac event three to tendays before CMR evaluation (rhythm disturbances, angina pectoris, shortness of breath). Left/right ventricular (LV/RV) volumes and ejection fractions (EF), as well as LV mass, the T2-signal ratio, early/late gadolinium enhancement (EGE/LGE), native/post-contrast T1-mapping, T2-mapping and extracellular volume fraction (ECV) were compared between the event and no-event groups. Results: No differences were identified in LV/RV volumes/EF/mass. In logistic regression analyses, independent predictors of belonging to the event group were EGE (odds ratio (95% CI): 1.55 (1.06-2.26), p = 0.024), LGE (1.81 (1.23-2.67), p = 0.003), T2 mapping (1.20 (1.06-1.36), p = 0.004) and native/post-contrast T1 mapping (1.17 (1.04-1.32), p = 0.007 and 0.86 (0.75-0.98), p = 0.025). At a median follow-up of~1.2 years, 42% vs. 11% of the event/no-event group respectively reached a combined endpoint of event occurrence/recurrence or cardiovascular mortality. Of the independent predictors resulting from logistic regression analyses, only LGE (hazard ratio (95% CI): 1.20 (1.11-1.30), p < 0.001), T2-mapping (1.07 (1.01-1.14), p = 0.025) and native T1-mapping (1.08 (1.01-1.15), p = 0.017) independently predicted the combined endpoint. Conclusions: A normal echocardiogram does not preclude myocardial lesions in diffuse SSc patients, which can be detected by CMR especially in symptomatic patients.

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“…The United Kingdom consensus position is to monitor the variable aspects of the DETECT protocol (ECG axis, urate, NTproBNP, and DLCO) for significant changes and re-catheterize if these suggest progression. 16 There are no convincing data to support screening in other CTDs-a study is currently underway to see if a screening program can be developed for systemic lupus erythematosus (SLE) where there is less certainty that the frequency of PH is sufficient to render such a program useful. 17 Screening clearly should not be used in RA or Sjogren's syndrome where the prevalence is far too low to justify such effort.…”

Section: Screening For Pahmentioning

confidence: 99%

Pulmonary Hypertension Associated with Connective Tissue Disease

Fayed

Coghlan

2019

Semin Respir Crit Care Med

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Pulmonary hypertension (PH) is common in most forms of connective tissue disease (CTD); the prevalent type of PH depends on the particular CTD. Thus, pulmonary arterial hypertension (PAH) is dominantly associated with scleroderma, while postcapillary PH is most common in rheumatoid arthritis and lung disease-associated PH is typically found in myositis and sarcoidosis.Considerable expertise is required to identify, diagnose, and manage CTD-PH, as the primary physicians providing the majority of care for this population, rheumatologists, need a good working knowledge of CTD-PH, its rather subtle presentation, and how to access the necessary investigations to screen for and identify patients with PH. The role of the rheumatologist does not stop at diagnosis; in some conditions such as lupus, optimizing immunosuppression is key to the management of PH, and unlike simple idiopathic PAH, the natural history of CTD-PH is often punctuated by complications of the CTD rather than just events due to progression of PH or therapy-related adverse events.The aim of this article is to provide an overview of all forms of CTD-PH, and to provide an easy reference source on current best practice.

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Consensus best practice pathway of the UK Systemic Sclerosis Study group: management of cardiac disease in systemic sclerosis

Abstract: The physician should be alert to the possibility of cardiac disease in SSc; it is best managed within a multidisciplinary team including both rheumatologists and cardiologists. This pathway provides a reference for all physicians managing patients with SSc.

Cited by 87 publications

References 41 publications

Effect of rituximab on heart involvement in systemic sclerosis

Effect of rituximab on heart involvement in systemic sclerosis

Cardiovascular Magnetic Resonance Identifies High-Risk Systemic Sclerosis Patients with Normal Echocardiograms and Provides Incremental Prognostic Value

Pulmonary Hypertension Associated with Connective Tissue Disease

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