Avaliação do envolvimento cardíaco por ressonância magnética cardíaca e correlação com a vasculopatia periférica em pacientes com esclerose sistêmica
Data
2024-09-30
Autores
Martins, Lucas Victória de Oliveira 
Orientadores
Kayser, Cristiane
Tipo
Tese de doutorado
Título da Revista
ISSN da Revista
Título de Volume
Resumo
Introdução/Objetivo: A esclerose sistêmica (ES) é uma doença reumática imunomediada cuja fisiopatogênese é composta por vasculopatia, autoimunidade com inflamação e fibrose generalizada com manifestações clínicas como fenômeno de Raynaud por envolvimento da microcirculação periférica e envolvimento cardíaco (ECES). O envolvimento da microcirculação periférica pode ser avaliado pela capilaroscopia periungueal (CPU) que visualiza alterações anatômicas da rede capilar relacionadas à ES, como dilatação e desvascularização capilar. O EC-ES pode ser avaliado por diferentes métodos, incluindo a ressonância magnética cardíaca (RMC) que avalia morfologia e função cardíaca e, especialmente, alterações cardíacas associados à vasculopatia, inflamação ou fibrose da ES. O objetivo deste estudo foi avaliar alterações na RMC com protocolo de estímulo frio em pacientes com ES e controles e correlacionar alterações do EC-ES por RMC com alterações anatômicas da microcirculação periférica por CPU. Métodos: 40 pacientes com ES e 10 controles saudáveis pareados por idade e sexo realizaram RMC com protocolo de estresse térmico por estímulo frio. Foram avaliados morfologia e função cardíaca, global longitudinal strain de ventrículo esquerdo (GLS-VE) e ventrículo direito (GLS-VD), perfusão qualitativa por defeitos perfusionais pós-frio, perfusão quantitativa por
myocardial blood flow (MBF) em repouso e estresse, myocardial perfusion reserve (MPR) (razão entre MBF estresse por MBF repouso), fibrose miocárdica por lesões de realce tardio (RT) e envolvimento pericárdico. Os pacientes com ES realizaram CPU para avaliação da densidade capilar avaliada pelo número médio de alças capilares/mm, escore de desvascularização e padrão SD (scleroderma) tardio. Resultados: Os pacientes com ES tinham idade média de 51 ± 12,0 anos, em sua maioria do sexo feminino (95%), e com forma difusa (52,5%). Trinta (75%) pacientes tinham CPU com padrão não-SD tardio e 10 (25%) com padrão SD tardio. Não houve diferença na comparação dos parâmetros de morfologia e função cardíaca, GLS-VE, GLS-VD, MBF repouso, MBF estresse e MPR na RMC entre pacientes com ES e controles. A maioria dos pacientes com ES (55%) apresentaram alteração cardíaca por EC-ES na RMC. Foram descritas alterações associadas à vasculopatia em 15/40 (37,5%), como defeitos perfusionais induzidos por frio (5%) e MPR < 1,0 (13/34, 38,2%), e associadas à fibrose em 10/40 (20%), como GLS-VE < 14%, RT e falência biventricular em 12,5%, 10% e 10%, respectivamente, dos pacientes. O grupo SD tardio apresentou maior massa indexada de VE (p <0,01), menor GLS-VE (p<0,01), maior frequência de GLS-VE < 14% (p=0,01), de derrame pericárdico
moderado/importante (p=0,04) e de RT (p=0,01) na RMC quando comparados com o grupo não-SD tardio. Em relação a alterações relacionadas à perfusão, escore de desvascularização foi significantemente maior nos pacientes com MPR < 1,0 em comparação aqueles sem MPR < 1,0 (1,77 ± 1,04 versus 1,00 ± 1,00, respectivamente; p=0,04). O padrão SD tardio e número médio de alças capilares/mm < 3,0 foram associados com um maior risco para alterações relacionadas à fibrose na RMC (OR = 9,00; IC95% 1,61 – 50,3 e OR = 15,0; IC95% 2,05 – 110, respectivamente). O padrão SD tardio foi também associado a um maior risco para derrame pericárdico moderado/importante (OR = 12,4; IC95% 1,12 – 138). Conclusões: Alterações cardíacas por EC-ES na RMC são frequentes em pacientes com ES. A presença de maiores graus de desvascularização capilar que é característica do padrão SD tardio e número médio de alças capilares/mm reduzido foi associada com diversas alterações na RMC relacionadas à vasculopatia ou fibrose no EC-ES. Alterações na microcirculação periférica avaliadas pela CPU podem refletir o EC-ES, desta maneira, sugerindo que a CPU possa ser um potencial biomarcador para EC-ES.
Introduction/Objective: Systemic sclerosis (SSc) is an immune-mediated rheumatic disease whose physiopathogenesis is composed of vasculopathy, autoimmunity with inflammation, and generalized fibrosis with clinical manifestations as Raynaud's phenomenon due to involvement of the peripheral microcirculation and heart involvement (SSc-HI). Peripheral microcirculation involvement can be assessed by nailfold capillaroscopy (NFC), which visualizes SSc-related anatomical changes in the capillary network, such as capillary dilation and devascularization. SSc-HI can be evaluated by different methods, including cardiac magnetic resonance (CMR), which assesses cardiac morphology and function and, especially, cardiac abnormalities of SSc-related vasculopathy, inflammation, or fibrosis. The aim of this study was to evaluate CMR changes with a cold stress protocol in SSc patients and controls and to correlate SSc-HI changes by CMR with anatomic changes in the peripheral microcirculation by NFC. Methods: Forty SSc patients and 10 age- and sex-matched healthy controls underwent cold stress protocol CMR. Cardiac morphology and function, global longitudinal strain of the left ventricle (LV-GLS) and right ventricle (RVGLS), qualitative perfusion by cold-induced perfusion defects, quantitative perfusion by myocardial blood flow (MBF) at rest and stress, myocardial perfusion reserve (MPR) (ratio between MBF at stress and MBF at rest), myocardial fibrosis by late gadolinium enhancement (LGE) lesions, and pericardial involvement were evaluated. SSc patients underwent NFC to evaluate capillary density by the mean number of capillary loops/mm, avascular score, and late SD (scleroderma) pattern. Results: SSc patients had a mean age of 51 ± 12.0 years, most were female (95%), and had diffuse form (52.5%). Thirty (75%) patients had NFC with a late non-SD pattern and 10 (25%) had late SD pattern. There was no difference in the comparison of cardiac morphology and function parameters, LV-GLS, RV-GLS, MBF rest, MBF stress, and MPR on CMR between SSc patients and controls. Most SSc patients (55%) had SSc-HI abnormalities on CMR. Alterations associated with vasculopathy were described in 15/40 (37.5%) of the patients, including cold-induced perfusion defects (5%) and MPR < 1.0 (13/34, 38.2%), and associated with fibrosis in 10/40 (20%), including LV-GLS < 14%, LGE lesions, and biventricular failure in 12.5%, 10%, and 10% of the patients, respectively. The late SD group had higher LV indexed mass (p <0.01), lower LV-GLS (p <0.01), a higher frequency of LV-GLS <14% (p = 0.01), moderate/severe pericardial effusion (p = 0.04), and LGE lesions (p = 0.01) on CMR when compared with the nonlate SD group. Regarding perfusion-related changes, avascular score was significantly higher in patients with MPR <1.0 compared with those without MPR <1.0 (1.77 ± 1.04 versus 1.00 ± 1.00, respectively; p = 0.04). The late SD pattern and a mean number of capillary loops/mm < 3.0 were associated with a higher risk for fibrosis-related changes on CMR (OR = 9.00; 95%CI 1.61–50.3 and OR = 15.0; 95%CI 2.05–110, respectively). The late SD pattern was also associated with a higher risk for oderate/severe pericardial effusion (OR = 12.4; 95%CI 1.12–138). Conclusions: Cardiac changes due to SSc-HI on CMR are frequent in SSc patients. The presence of higher degrees of capillary devascularization that is characteristic of the late SD pattern and reduced mean number of loops/mm were associated with several CMR changes related to vasculopathy or fibrosis in SSc-HI. Peripheral microcirculation abnormalities assessed by NFC may reflect SSc-HI, thus suggesting that NFC may be a potential biomarker for CS-ES.
Introduction/Objective: Systemic sclerosis (SSc) is an immune-mediated rheumatic disease whose physiopathogenesis is composed of vasculopathy, autoimmunity with inflammation, and generalized fibrosis with clinical manifestations as Raynaud's phenomenon due to involvement of the peripheral microcirculation and heart involvement (SSc-HI). Peripheral microcirculation involvement can be assessed by nailfold capillaroscopy (NFC), which visualizes SSc-related anatomical changes in the capillary network, such as capillary dilation and devascularization. SSc-HI can be evaluated by different methods, including cardiac magnetic resonance (CMR), which assesses cardiac morphology and function and, especially, cardiac abnormalities of SSc-related vasculopathy, inflammation, or fibrosis. The aim of this study was to evaluate CMR changes with a cold stress protocol in SSc patients and controls and to correlate SSc-HI changes by CMR with anatomic changes in the peripheral microcirculation by NFC. Methods: Forty SSc patients and 10 age- and sex-matched healthy controls underwent cold stress protocol CMR. Cardiac morphology and function, global longitudinal strain of the left ventricle (LV-GLS) and right ventricle (RVGLS), qualitative perfusion by cold-induced perfusion defects, quantitative perfusion by myocardial blood flow (MBF) at rest and stress, myocardial perfusion reserve (MPR) (ratio between MBF at stress and MBF at rest), myocardial fibrosis by late gadolinium enhancement (LGE) lesions, and pericardial involvement were evaluated. SSc patients underwent NFC to evaluate capillary density by the mean number of capillary loops/mm, avascular score, and late SD (scleroderma) pattern. Results: SSc patients had a mean age of 51 ± 12.0 years, most were female (95%), and had diffuse form (52.5%). Thirty (75%) patients had NFC with a late non-SD pattern and 10 (25%) had late SD pattern. There was no difference in the comparison of cardiac morphology and function parameters, LV-GLS, RV-GLS, MBF rest, MBF stress, and MPR on CMR between SSc patients and controls. Most SSc patients (55%) had SSc-HI abnormalities on CMR. Alterations associated with vasculopathy were described in 15/40 (37.5%) of the patients, including cold-induced perfusion defects (5%) and MPR < 1.0 (13/34, 38.2%), and associated with fibrosis in 10/40 (20%), including LV-GLS < 14%, LGE lesions, and biventricular failure in 12.5%, 10%, and 10% of the patients, respectively. The late SD group had higher LV indexed mass (p <0.01), lower LV-GLS (p <0.01), a higher frequency of LV-GLS <14% (p = 0.01), moderate/severe pericardial effusion (p = 0.04), and LGE lesions (p = 0.01) on CMR when compared with the nonlate SD group. Regarding perfusion-related changes, avascular score was significantly higher in patients with MPR <1.0 compared with those without MPR <1.0 (1.77 ± 1.04 versus 1.00 ± 1.00, respectively; p = 0.04). The late SD pattern and a mean number of capillary loops/mm < 3.0 were associated with a higher risk for fibrosis-related changes on CMR (OR = 9.00; 95%CI 1.61–50.3 and OR = 15.0; 95%CI 2.05–110, respectively). The late SD pattern was also associated with a higher risk for oderate/severe pericardial effusion (OR = 12.4; 95%CI 1.12–138). Conclusions: Cardiac changes due to SSc-HI on CMR are frequent in SSc patients. The presence of higher degrees of capillary devascularization that is characteristic of the late SD pattern and reduced mean number of loops/mm were associated with several CMR changes related to vasculopathy or fibrosis in SSc-HI. Peripheral microcirculation abnormalities assessed by NFC may reflect SSc-HI, thus suggesting that NFC may be a potential biomarker for CS-ES.