RESUMEN
Abstract 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.
Resumen Introducción: La afección pulmonar en la esclerosis sistémica juvenil (ESJ) es rara en niños y contribuye a la morbimortalidad. El objetivo de este estudio fue describir los hallazgos de función pulmonar, clínicos, radiológicos y tomográficos en la ESJ. Métodos: Se incluyeron pacientes con ESJ de 5-14 años de edad. Se evaluaron las características clínicas, funcionales y de imagen. No se incluyeron pacientes con enfermedades pulmonares no asociadas con ESJ en el momento del estudio: enfermedad mixta del tejido conectivo, síndrome de superposición o insuficiencia cardiopulmonar aguda. Se realizaron exploración física, electrocardiograma, espirometría, radiografía de tórax, tomografía computarizada de alta resolución de tórax, ecocardiografía, pruebas de función pulmonar y prueba de caminata de 6 minutos (PC6M). Se utilizó estadística descriptiva para el análisis de los datos. Resultados: Se estudiaron 15 pacientes con las siguientes características: mediana de edad, 11 años; mediana desde el inicio de los síntomas, 6 años; y mediana desde el diagnóstico de ESJ y hallazgo de afección pulmonar, 2 años. Se detectó enfermedad pulmonar en el 73%. La enfermedad pulmonar intersticial (EPI) fue la afección más común (67%) y se encontró hipertensión pulmonar en el 6.6%. La PC6M fue positiva en el 26.6%, y la capacidad vital forzada resultó anormal en el 26.6%. Cuatro pacientes no presentaron afección pulmonar. Conclusiones: La afección pulmonar más frecuente en la ESJ fue la EPI. La detección temprana de ESJ y la monitorización pulmonar periódica son obligatorias para evitar más complicaciones una vez diagnosticada la ESJ.
RESUMEN
Scleroderma is a set of rare connective tissue diseases of unknown etiology. It is characterized by thickening and hardening of the skin. Scleroderma is divided into two main subgroups: systemic and localized. The systemic form, also known as systemic sclerosis, involves diffuse skin involvement associated with fibrotic changes in internal organs. Juvenile localized scleroderma is a more common entity and is usually confined to a specific region of the body with no internal organ involvement. Therapeutics are divided into three main subgroups for juvenile systemic sclerosis: antifibrotics, anti-inflammatories, and vasodilators. For localized disease, anti-inflammatories, vitamin D analogues, and UV irradiation have been investigated. The rarity of scleroderma in children and the self-limiting nature of the disease together make randomized controlled trials very difficult. Therefore, most data on therapeutic modalities for this condition have to be extrapolated from studies conducted on adults. International cooperation, following a standardized operation protocol, is needed to validate these and future interventions such as autologous stem cell transplant and cytokine-directed therapies.