Pulmonary involvement in patients with juvenile systemic sclerosis.

Autor:	Céspedes-Cruz AI; Departamento de Reumatología Pediátrica, Hospital de Especialidades Centro Médico La Raza, Instituto Mexicano del Seguro Social (IMSS), Mexico City. Mexico.; Universidad Nacional Autónoma de México, Mexico City. Mexico., Carranza-Muleiro RA; División de Investigación en Salud, Hospital de Especialidades Centro Médico La Raza, IMSS, Mexico City. Mexico.; Sección de Estudios de Posgrado e Investigación, Escuela Superior de Medicina, Instituto Politécnico Nacional, Mexico City. Mexico., López-Rojas EL; Hospital de Gineco-Pediatría 48, IMSS, León, Guanajuato. Mexico., Cruz-Domínguez MP; Universidad Nacional Autónoma de México, Mexico City. Mexico.; División de Investigación en Salud, Hospital de Especialidades Centro Médico La Raza, IMSS, Mexico City. Mexico., Espinosa-Gan H; Departamento de Cardiología Pediátrica, Hospital de Especialidades Centro Médico La Raza, IMSS, Mexico City. Mexico., Ramírez-Pérez J; Departamento de Radiología, Hospital de Especialidades Centro Médico La Raza, IMSS, Mexico City. Mexico., Moreno-Martínez JM; Departamento de Radiología, Hospital de Especialidades Centro Médico La Raza, IMSS, Mexico City. Mexico., Moysén-Ramírez S; Departamento de Neumología, Hospital de Especialidades Centro Médico La Raza, IMSS, Mexico City. Mexico., Zeferino-Cruz M; Hospital General Gaudencio González Garza, Centro Médico La Raza, IMSS, Mexico City. Mexico., Torres-Jiménez AR; Hospital General Gaudencio González Garza, Centro Médico La Raza, IMSS, Mexico City. Mexico., Ordoñez-González I; Universidad Veracruzana, Campus Veracruz, Veracruz, Veracruz. Mexico., Medina G; Universidad Nacional Autónoma de México, Mexico City. Mexico.; División de Investigación en Salud, Hospital de Especialidades Centro Médico La Raza, IMSS, Mexico City. Mexico.; Unidad de Investigación en Medicina Traslacional, Hospital de Especialidades Centro Médico La Raza, IMSS, Mexico City. Mexico.
Jazyk:	angličtina
Zdroj:	Boletin medico del Hospital Infantil de Mexico [Bol Med Hosp Infant Mex] 2021; Vol. 78 (5), pp. 385-394.
DOI:	10.24875/BMHIM.20000301
Abstrakt:	Background: Pulmonary involvement in juvenile systemic sclerosis (JSSc) is rare in children and contributes to morbimortality. This study aimed to describe the pulmonary function and clinical, radiologic, and tomographic findings in JSSc. Methods: Patients with JSSc between 5-14 years of age were included. Clinical, functional, and imaging characteristics were assessed. Patients were excluded if they showed lung disease not associated with JSSc: mixed connective tissue disease, overlap syndrome, or acute cardiopulmonary failure at the time of the study. All patients underwent physical examination, electrocardiogram, spirometry, chest X-ray, high-resolution computed tomography (HRCT) of the chest, echocardiography, lung function tests, and the 6-minute walk test (6-MWT). Descriptive statistics were employed for data analysis. Results: We studied 15 patients with the following characteristics: median age, 11 years; median since symptoms onset, 6 years; median since JSSc diagnosis and the finding of pulmonary involvement, 2 years. Lung disease was detected in 73%, interstitial lung disease (ILD) the most common affection (67%); pulmonary hypertension was found in 6.6%. 6-MWT was positive in 26.6%, forced vital capacity (FVC) was abnormal in 26.6%. No pulmonary involvement was found in four patients. Conclusions: The most frequent pulmonary affection in JSSc was ILD. Thus, early JSSc detection and periodic lung monitoring are mandatory to avoid further complications once JSSc is diagnosed. (Copyright: © 2021 Permanyer.)
Databáze:	MEDLINE
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