Systemic sclerosis (SSc) is an autoimmune rheumatic disease characterized by alterations in the immune response and a vasculopathy affecting the microcirculation and small-caliber arteries with consequent fibrosis at the level of the skin, the musculoskeletal system and various internal organs, including lungs, heart and kidneys. Cardiac involvement can be primary or secondary to pulmonary arterial hypertension and kidney disease. Unfortunately, all laboratory and imaging parameters used to evaluate cardiac involvement are able to identify it only in the advanced stage of the pathology; we are therefore looking for a parameter to early recognize the heart involvement during systemic sclerosis. One of the possible markers currently being studied is the global longitudinal strain (GLS), which can be calculated with speckle-tracking echocardiography and assesses the movement of an entire cardiac chamber. The data collected from numerous studies suggest its usefulness, given that unlike the normal parameters used for the evaluation of cardiac function, in both ventricles there are statistically significant differences by comparing the GLS of the patients with that of the controls. These data are certainly consistent with the possibility of using GLS as a marker of subclinical cardiac damage from SSc, but it is not yet known if this form of subclinical cardiomyopathy remains stable over time or if with the progress of the underlying disease it will also meet it to a worsening. The aim of this work is therefore to identify the possible progression of biventricular subclinical cardiac involvement in patients with systemic sclerosis using GLS. An observational study was conducted on 72 patients with systemic sclerosis; for each patient, data were collected regarding the characteristics of the pathology, the drugs taken and the involvement of organs such as esophagus and lungs. On echocardiography, parameters related to two-dimensional images, Doppler and speckle-tracking were considered. The five patients with the best GLS value in the left ventricle and the five patients with the worst value were then studied with cardiac magnetic resonance imaging. Echocardiographic parameters did not statistically significantly differ between baseline and follow-up, and were within the normal range. The GLS of both ventricles, however, was already affected at baseline, and worsened significantly during follow-up; the greatest progression occurred in the endocardial layer of both ventricles. At cardio-MRI, there were no differences in terms of volumes, ejection fraction, range and masses between the patients with the best GLS and those with the worst GLS. The main evidence of this study is that SSc cardiomyopathy progresses over time and GLS is an excellent parameter for following its progress during follow-up. GLS is also a good predictor of the risk of incurring clear manifestations of the disease, such as pulmonary hypertension. The data collected support the hypothesis that the pathophysiology of SSc-related subclinical heart damage is linked to microvascular dysfunctions of the coronary circulation, due to the greater strain impairment in the sub-endocardial layers and the absence of cardiac fibrosis, as seen in cardio-MRI. It is therefore possible to conclude that the subclinical cardiac impairment of patients with SSc progresses over time, involving both ventricles. The GLS allows to evaluate this involvement in an effective and non-invasive way, resulting a fundamental marker of cardiac damage to be considered in the diagnosis and follow-up.
La sclerosi sistemica (SSc) è una patologia autoimmune reumatica caratterizzata da alterazioni della risposta immunitaria e da una vasculopatia che riguarda il microcircolo e le arterie di piccolo calibro con conseguente fibrosi a livello della cute, dell’apparato muscolo-scheletrico e di vari organi interni, tra i quali polmoni, cuore e reni. Il coinvolgimento cardiaco può essere primario o secondario all’ipertensione arteriosa polmonare ed alla patologia renale. Sfortunatamente, tutti i parametri laboratoristici e di imaging utilizzati per valutare il coinvolgimento cardiaco sono in grado di individuarlo solo nella fase avanzata della patologia; si è quindi alla ricerca di un parametro per riconoscere precocemente un coinvolgimento del cuore in corso di sclerosi sistemica. Uno dei possibili marcatori attualmente in studio è il global longitudinal strain (GLS), calcolabile con l’ecocardiografia speckle-tracking, il quale valuta il movimento di un’intera camera cardiaca. I dati raccolti da numerosi studi ne suggeriscono l’utilità, dato che a differenza dei normali parametri utilizzati per la valutazione della funzione cardiaca, in entrambi i ventricoli ci sono differenze statisticamente significative confrontando il GLS dei pazienti con quello dei controlli. Tali dati sono sicuramente coerenti con la possibilità di utilizzare il GLS come marcatore di danno cardiaco subclinico da SSc, ma non è ancora noto se tale forma di cardiomiopatia subclinica rimanga stabile nel corso del tempo o se col progredire della malattia di base vada incontro anch’essa ad un peggioramento. Scopo di questo lavoro è pertanto individuare l’eventuale progressione del coinvolgimento cardiaco subclinico biventricolare in pazienti affetti da sclerosi sistemica utilizzando il GLS. È stato condotto uno studio osservazionale su 72 pazienti con sclerosi sistemica; per ogni paziente, sono stati raccolti dati riguardo le caratteristiche della patologia, i farmaci assunti e il coinvolgimento di organi come esofago e polmone. All’ecocardiografia, sono stati considerati parametri legati alle immagini bidimensionali, al Doppler ed allo speckle-tracking. I cinque pazienti con il miglior valore di GLS del ventricolo sinistro e i cinque con il valore peggiore sono poi stati studiati con risonanza magnetica cardiaca. I parametri ecocardiografici non differivano in maniera statisticamente significativa tra il tempo zero ed il follow-up, ed erano all’interno dell’intervallo di normalità. Il GLS di entrambi i ventricoli, invece, era già intaccato al basale, ed è peggiorato in maniera significativa durante il follow-up; la maggiore progressione si aveva nello strato endocardico di entrambi i ventricoli. Alla cardio-RM, non sono state riscontrate differenze in termini di volumi, frazione di eiezione, gittata e masse tra i pazienti col GLS migliore e quelli col GLS peggiore. L’evidenza principale di questo studio è che la cardiomiopatia da SSc progredisce nel corso del tempo ed il GLS è un ottimo parametro per seguirne l’andamento durante il follow-up. Il GLS è anche un buon predittore del rischio di incorrere in chiare manifestazioni della malattia, come ad esempio l’ipertensione polmonare. I dati raccolti supportano l’ipotesi che la fisiopatologia del danno cardiaco subclinico da SSc sia legata a disfunzioni microvascolari del circolo coronarico, per la maggiore compromissione dello strain negli strati sub-endocardici e l’assenza di fibrosi cardiaca, come visto alla cardio-RM. È possibile quindi concludere che il coinvolgimento cardiaco subclinico dei pazienti con SSc progredisce nel corso del tempo, coinvolgendo entrambi i ventricoli. Il GLS permette di valutare tale interessamento in maniera efficace e non invasiva, risultando un marker fondamentale di danno cardiaco da considerare nella diagnosi e nel follow-up.
Progressione del danno cardiaco subclinico in pazienti affetti da sclerosi sistemica
GIULIANI, LORENZO
2019/2020
Abstract
Systemic sclerosis (SSc) is an autoimmune rheumatic disease characterized by alterations in the immune response and a vasculopathy affecting the microcirculation and small-caliber arteries with consequent fibrosis at the level of the skin, the musculoskeletal system and various internal organs, including lungs, heart and kidneys. Cardiac involvement can be primary or secondary to pulmonary arterial hypertension and kidney disease. Unfortunately, all laboratory and imaging parameters used to evaluate cardiac involvement are able to identify it only in the advanced stage of the pathology; we are therefore looking for a parameter to early recognize the heart involvement during systemic sclerosis. One of the possible markers currently being studied is the global longitudinal strain (GLS), which can be calculated with speckle-tracking echocardiography and assesses the movement of an entire cardiac chamber. The data collected from numerous studies suggest its usefulness, given that unlike the normal parameters used for the evaluation of cardiac function, in both ventricles there are statistically significant differences by comparing the GLS of the patients with that of the controls. These data are certainly consistent with the possibility of using GLS as a marker of subclinical cardiac damage from SSc, but it is not yet known if this form of subclinical cardiomyopathy remains stable over time or if with the progress of the underlying disease it will also meet it to a worsening. The aim of this work is therefore to identify the possible progression of biventricular subclinical cardiac involvement in patients with systemic sclerosis using GLS. An observational study was conducted on 72 patients with systemic sclerosis; for each patient, data were collected regarding the characteristics of the pathology, the drugs taken and the involvement of organs such as esophagus and lungs. On echocardiography, parameters related to two-dimensional images, Doppler and speckle-tracking were considered. The five patients with the best GLS value in the left ventricle and the five patients with the worst value were then studied with cardiac magnetic resonance imaging. Echocardiographic parameters did not statistically significantly differ between baseline and follow-up, and were within the normal range. The GLS of both ventricles, however, was already affected at baseline, and worsened significantly during follow-up; the greatest progression occurred in the endocardial layer of both ventricles. At cardio-MRI, there were no differences in terms of volumes, ejection fraction, range and masses between the patients with the best GLS and those with the worst GLS. The main evidence of this study is that SSc cardiomyopathy progresses over time and GLS is an excellent parameter for following its progress during follow-up. GLS is also a good predictor of the risk of incurring clear manifestations of the disease, such as pulmonary hypertension. The data collected support the hypothesis that the pathophysiology of SSc-related subclinical heart damage is linked to microvascular dysfunctions of the coronary circulation, due to the greater strain impairment in the sub-endocardial layers and the absence of cardiac fibrosis, as seen in cardio-MRI. It is therefore possible to conclude that the subclinical cardiac impairment of patients with SSc progresses over time, involving both ventricles. The GLS allows to evaluate this involvement in an effective and non-invasive way, resulting a fundamental marker of cardiac damage to be considered in the diagnosis and follow-up.| File | Dimensione | Formato | |
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https://hdl.handle.net/20.500.12075/2010