Factors associated with pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc)

Jiang, Yuxuan; Turk, Matthew; Pope, Janet

doi:10.1016/j.autrev.2020.102602

Cited by 30 publications

(22 citation statements)

References 43 publications

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“…Na forma limitada, menor escore de Rodnan-m, gênero feminino e raça caucasiana são fatores protetores quanto a DPI e mortalidade 11,13 , o que seria uma possível explicação para o longo tempo de doença observado em nossos pacientes. A HAP é responsável por mais da metade dos óbitos na ES, sobretudo na forma limitada, 93% dos quais ocorrem em até quatro anos do diagnóstico 14,15 , sendo este o ponto de maior vulnerabilidade da população que foi estudada nesse trabalho. Quanto à questão étnica, há um viés populacional, por conta da miscigenação observada na população brasileira.…”

Section: Discussionunclassified

Síndrome De Sobreposição Esclerose Sistêmica E Lúpus Eritematoso Sistêmico: Perfil Epidemiológico

Rebouças

Skare

2022

RMP

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RESUMO – Racional: Esclerose sistêmica (ES) e lúpus eritematoso sistêmico (LES) são doenças difusas do tecido conjuntivo que, quando agregadas, geram a síndrome de sobreposição (SP ES-LES), condição ainda não claramente caracterizada do ponto de vista clínico e epidemiológico. Objetivo: Caracterizar clínica e epidemiologicamente pacientes com SP ES-LES. Método: Trata-se de um estudo retrospectivo, descritivo de análise de prontuários. Coletaram-se dados demográficos, clínicos, laboratoriais e exames de imagens de pacientes com SP ES-LES.Resultados: O perfil encontrado foi o de mulher, jovem, caucasiana, não tabagista e com forma de limitada de ES. Foram comuns: fenômeno de Raynaud; acometimento gastroesofágico, pulmonar e cutâneo; anemia; linfopenia; FAN pontilhado grosso; anti-RNP; anti-Ro e anti-Sm. Nunca ter fumado foi achado protetor para serosite e lesões discoides, como também foram significativas associações anti-Scl-70 com lúpus discoide, anti-La com síndrome de Sjögren e anti-DNA com úlceras digitais. Conclusão: A SP ES-LES é multissistêmica, com aspectos clínicos predominantes de ES. Devido à grande prevalência de envolvimento pulmonar nesses pacientes, justifica-se o rastreamento de possíveis complicações.

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Section: Discussionunclassified

Síndrome De Sobreposição Esclerose Sistêmica E Lúpus Eritematoso Sistêmico: Perfil Epidemiológico

Rebouças

Skare

2022

RMP

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“…Notably, pulmonary arterial hypertension is one of the most severe and potentially life-threatening complications in SSc [ 37 , 38 ]. The VSMCs’ activity also plays an important role in the development of pulmonary arterial hypertension through the same conversion from a contractile phenotype to a synthetic one [ 28 , 39 ].…”

Section: Smooth Muscle Involvementmentioning

confidence: 99%

The Involvement of Smooth Muscle, Striated Muscle, and the Myocardium in Scleroderma: A Review

Bratoiu

Burlui

Cardoneanu

et al. 2022

IJMS

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Systemic sclerosis (SSc) is a complex autoimmune disease characterized by heterogeneous changes involving numerous organs and systems. The currently available data indicate that muscle injury (both smooth and striated muscles) is widespread and leads to significant morbidity, either directly or indirectly. From the consequences of smooth muscle involvement in the tunica media of blood vessels or at the level of the digestive tract, to skeletal myopathy (which may be interpreted strictly in the context of SSc, or as an overlap with idiopathic inflammatory myopathies), muscular injury in scleroderma translates to a number of notable clinical manifestations. Heart involvement in SSc is heterogenous depending on the definition used in the various studies. The majority of SSc patients experience a silent form of cardiac disease. The present review summarizes certain important features of myocardial, as well as smooth and skeletal muscle involvement in SSc. Further research is needed to fully describe and understand the pathogenic pathways and the implications of muscle involvement in scleroderma.

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“…PH can develop in the course of most CTDs but is by far the most common in SSc and SScrelated diseases such as MCTD and some forms of myositis [27]. The most common form is PAH, which affects approximately 8%-15% of SSc patients [27,28]. The multi-organ nature of CTDs also increases susceptibility to other forms of PH.…”

Section: The Role Of the Rheumatologist In The Diagnosis Of Pulmonary Arterial Hypertensionmentioning

confidence: 99%

“…The inclusion of other clinical, functional, and biochemical parameters in screening may improve the detection of PAH in patients with CTD [21,28,[31][32][33]. The DETECT algorithm, based on the analysis of the aforementioned study, is designed to determine the risk of PAH in patients with SSc [28]. As shown in Figure 1, The DETECT algorithm includes two steps: the first is to assess the need for an echo and the second is to qualify for right heart catheterization.…”

Section: Registry To Evaluate Early and Long-term Pulmonary Arterial Hypertension Diseasementioning

confidence: 99%

An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation and the Polish Society for Rheumatology on the diagnosis and treatment of pulmonary hypertension in patients with connective tissue disease

et al. 2021

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Factors associated with pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc)

Cited by 30 publications

References 43 publications

Síndrome De Sobreposição Esclerose Sistêmica E Lúpus Eritematoso Sistêmico: Perfil Epidemiológico

Síndrome De Sobreposição Esclerose Sistêmica E Lúpus Eritematoso Sistêmico: Perfil Epidemiológico

The Involvement of Smooth Muscle, Striated Muscle, and the Myocardium in Scleroderma: A Review

An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation and the Polish Society for Rheumatology on the diagnosis and treatment of pulmonary hypertension in patients with connective tissue disease

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