Prognostic and functional importance of both overt and subclinical left ventricular systolic dysfunction in systemic sclerosis

Fairley, Jessica L.; Hansen, Dylan; Proudman, Susanna; Sahhar, Joanne; Ngian, Gene-Siew; Walker, Jenny; Host, Lauren V.; La Gerche, André; Prior, David; Burns, Andrew; Morrisroe, Kathleen; Stevens, Wendy; Nikpour, Mandana; Ross, Laura

doi:10.1016/j.semarthrit.2024.152443

Cited by 5 publications

(2 citation statements)

References 26 publications

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“…[ 1 ] SSc has the highest case-based mortality of all rheumatic diseases, and significantly reduced life expectancy. [ 2 ] SSc Heart Involvement (SHI) is associated with a poor prognosis, [ 3 , 4 ] but remains a poorly understood and under-recognised complication of SSc.…”

Section: Introductionmentioning

confidence: 99%

Cardiac magnetic resonance imaging in systemic sclerosis: Heart involvement in high-resolution

Fairley,

O’Rourke,

Puranik

et al. 2024

Rheumatology and Immunology Research

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Cardiac magnetic resonance imaging (CMR) is the gold-standard non-invasive method of assessing cardiac structure and function, including tissue characterisation. In systemic sclerosis (SSc), heart involvement (SHI) is a leading cause of mortality yet remains poorly understood. SHI is underestimated by conventional echocardiography, and CMR provides an important opportunity to better identify and quantify subtle myocardial changes including oedema and fibrosis. This review summarises current CMR techniques, the role of CMR in SSc and SHI, and the opportunities to further our understanding of its pathogenesis and management.

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Section: Introductionmentioning

confidence: 99%

Cardiac magnetic resonance imaging in systemic sclerosis: Heart involvement in high-resolution

Fairley,

O’Rourke,

Puranik

et al. 2024

Rheumatology and Immunology Research

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“…[ 10 ] While LV systolic dysfunction, another cardiac involvement in SSc, is associated with high mortality and diminished physical capability in patients with SSc. [ 11 ] According to the high mortality related to cardiac involvement, particularly LV systolic dysfunction among SSc patients, no predictor of LV systolic dysfunction in SSc has been defined. If predictors of LV systolic dysfunction are identified, interventions acting on these modifiable predictors might improve patient outcomes.…”

Section: Introductionmentioning

confidence: 99%

Clinical courses and predictors of left ventricular systolic dysfunction in systemic sclerosis: A cohort study

Werakiat,

Pussadhamma,

Mahakkanukrauh

et al. 2024

Rheumatology and Immunology Research

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Background and Objectives Left ventricular systolic dysfunction (LVSD) is a cardiac involvement that is the leading cause of death among patients with systemic sclerosis (SSc). We aimed to define the clinical course and predictors of LVSD among SSc patients. Methods We conducted a cohort study among adult patients with SSc who were followed up from 2013 to 2020. Semiparametric Cox regression analysis with robust clustering by cohort identification number was used to evaluate the predictors of LVSD. Results Among the 3, 987 person-years, LVSD was defined in 35 of 419 SSc patients for an incidence of 0.88 per 100 person-years. The median duration of the disease was 8.5 (interquartile range (IQR) 4.9–12.9) years. Every 1-point increase in the modified Rodnan skin score (mRSS) and salt and pepper skin were strong predictors of LVSD, with a respective adjusted hazard ratio (HR) of 1.05 and 3.17. During follow-up, 26 cases (74.3%) had unimproved LVSD. The strong predictors of the unimprovement of LVSD were every 1-point increase in mRSS (HR 1.05), every 1 mg increase in prednisolone treatment (HR 1.05), and every 1 U/L increase in creatine kinase (CK) (HR 1.001). Mycophenolate treatment was a protective factor against the unimprovement of LVSD in SSc (HR 0.15). Conclusions LVSD was frequently found in patients with diffuse cutaneous SSc, and in most cases, it remained unimproved during follow-up. High mRSS, steroid use, and high CK levels were predictors of unimproved LVSD, whereas mycophenolate treatment might prevent the progression of LVSD. Steroids should be prescribed with caution in patients with longer disease duration.

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Heart involvement in patients with systemic sclerosis—what have we learned about it in the last 5 years

Nadel,

Taborska

et al. 2024

Rheumatol Int

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This review provides a detailed examination of original research and previously published reviews regarding cardiovascular involvement in systemic sclerosis (SSc). Our study aims to evaluate the current understanding of SSc-associated heart involvement (SHI), focusing on its most prevalent forms, diagnostic methods and treatment options. A comprehensive search of PUBMED, Medline, Web of science, Scopus and DOAJ databases was conducted, involving articles published between January 2019 and August 2024, available in English, both original research and reviews. Additionally, the authors examined the references cited in the selected articles, reviewed relevant literature, and included key publications dating back to 2010. Systemic Sclerosis (SSc) is an autoimmune connective tissue disease characterized by skin and internal organs fibrosis with accompanying vasculopathy. SHI encompasses both primary and secondary cardiac disease with a prevalence rate of up to 39%. It constitutes one of the leading causes of death among affected individuals. Systemic sclerosis- primary heart involvement comprises a wide range of conditions including arrhythmias, heart failure, pericardial disease, valvular abnormalities, and myocardial inflammation. However, its subclinical course, often misinterpreted as other forms of cardiomyopathy, poses true diagnostic challenges, requiring diagnostic tools like transthoracic echocardiography with tissue Doppler echocardiography and cardiac magnetic resonance imaging. The review underscores the importance of SHI and a holistic approach to managing patients with systemic sclerosis. Furthermore, it emphasizes the need for further investigation into potential pathogenetic mechanisms and biomarkers crucial for targeted treatment to fully optimize recommendations for this patient subgroup.

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Prognostic and functional importance of both overt and subclinical left ventricular systolic dysfunction in systemic sclerosis

Cited by 5 publications

References 26 publications

Cardiac magnetic resonance imaging in systemic sclerosis: Heart involvement in high-resolution

Cardiac magnetic resonance imaging in systemic sclerosis: Heart involvement in high-resolution

Clinical courses and predictors of left ventricular systolic dysfunction in systemic sclerosis: A cohort study

Heart involvement in patients with systemic sclerosis—what have we learned about it in the last 5 years

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