Abstract

VELAZQUEZ-ORTIZ, José Osmar; PEREZ-PEREZ, Linda Fabiola  and  APARICIO-VERA, Luis Alberto. Pulmonary arterial hypertension in patients with juvenile dermatomyositis and systemic sclerosis in a third level pediatric center. Rev. parag. reumatol. [online]. 2023, vol.9, n.2, pp.58-63.  Epub Dec 30, 2023. ISSN 2413-4341.  https://doi.org/10.18004/rpr/2023.09.02.58.

INTRODUCTION: Pulmonary arterial hypertension may be secondary associated with connective tissue diseases. Among these diseases, systemic sclerosis and juvenile dermatomyositis predominate. MATERIALS AND METHODS: A retrospective, descriptive and cross-sectional study was carried out. All patients with a diagnosis of juvenile dermatomyositis and systemic sclerosis who attended our hospital were included. Pulmonary arterial pressure levels were subsequently verified by echocardiography. RESULTS: 58 patients were included, of which only 17 patients had a diagnostic echocardiography. Among them, two patients presented pulmonary arterial hypertension. CONCLUSION: Timely detection of pulmonary arterial hypertension in connective tissue diseases is essential. It is generally asymptomatic. It is necessary to adhere to the international protocol that suggests performing echocardiography in all patients with juvenile dermatomyositis and systemic sclerosis.

Keywords : Pulmonary arterial hypertension; juvenile dermatomyositis; systemic sclerosis; child..

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