Análisis descriptivo del compromiso pulmonar intersticial en pacientes con diagnóstico de esclerosis sistémica atendidos en consulta externa de Reumatología de los hospitales Maximiliano Peralta Jiménez y San Rafael de Alajuela, de la Caja Costarricesne del Seguro Social, del 1° enero del 2013 al 31 de marzo del 2019
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La esclerosis sistémica es una enfermedad crónica autoinmune, multiorgánica, poco frecuente, caracterizada por la inflamación y fibrosis principalmente cutánea, concomitante al daño endotelial y afección inflamatoria de vasos sanguíneos, tejidos osteomusculares y órganos internos. Se estima una incidencia en los Estados Unidos de 19 casos por 1.000.000 de habitantes/año y su prevalencia es de hasta 242 casos por 1.000.000 de adultos. Según la extensión del compromiso cutáneo, se puede clasificar la ES en difusa (ESD) o ES limitada (ESL). La primera se caracteriza por el endurecimiento generalizado de la piel, compromiso frecuente de órganos internos y presencia de anticuerpos antitopoisomerasa I (Scl-70), la que la convierte en la presentación clínica con peor pronóstico. La ESL se caracteriza por el endurecimiento de la piel a nivel distal en extremidades, cara y cuello, menor compromiso de órganos internos, presencia de anticuerpos anticentrómero y mejor pronóstico comparada con la ESD. El compromiso pulmonar intersticial se puede observar hasta en el 75% de los pacientes con esclerosis sistémica y sabemos que esta constituye la principal causa de muerte. Es necesario describir las características de nuestra población afectada. Para diagnosticar la enfermedad pulmonar intersticial es de gran importancia tener datos clínicos, perfil de anticuerpos asociados a la ES, imágenes obtenidas principalmente en TACAR de tórax y datos espirométricos como disminución en la CVF y DLCO. Se analizaron los datos de 109 pacientes con esclerosis sistémica con una edad media de 48 años. El 85.33% de los pacientes presentó ESL y 14,67% se asoció a ESD. Se determinó EPI en el 24% de los pacientes, los cuales presentaron un perfil de anticuerpos ACA y antiSCL 70 de 69,23% y 30,77%, respectivamente. Los pacientes con antiSCL 70 presentaron EPI en el 50% de los enfermos y, además, el compromiso pulmonar se observó en el 75% de ellos en los...
Inglés: Systemic sclerosis is a chronic autoimmune, multiorganic, rare disease, characterized by inflammation and mainly cutaneous fibrosis, associated with endothelial damage and inflammatory affection of blood vessels, osteomuscular tissues and internal organs. In addition an incidence of 19 cases per every 1,000,000 inhabitants is estimated in the United States with 19 cases per 1,000,000 inhabitants / year and its prevalence is up to 242 cases per 1,000,000 adults. According to the extent of skin involvement, SSc can be classified as diffuse (DSSc) or limited SSc (LSSc). The first is characterized by the generalized hardening of the skin, with frequent involvement of internal organs and the presence of antitopoisomerase I (Scl- 70) antibodies, which makes it the clinical presentation with the worst prognosis. SScL is characterized by toughening of the skin at distal level of extremities, such as face and neck, less involvement of damage in internal organs and presence of anticentromere antibodies with better prognosis compared with DSSc. Interstitial lung involvement can be observed in up to 75% of patients with systemic sclerosis and it's known to be the main cause of death. Therefore, it is necessary to describe the characteristics of our affected population. In order to diagnose interstitial lung disease, it is very important to have clinical data, the profile of antibodies associated with SSc, images obtained mainly by HRCT of thorax, and spirometric data for instance the decrease in FVC and DLCO. Data of 109 patients with systemic sclerosis has been analysed with an average age of 48 years. The 85.33% of the patients presented LSSc and the other 14.67% was associated with SScD. ILD was determined in 24% of the patients, who presented an ACA and antiSCL 70 antibody profile of 69.23% and 30.77%, respectively. Patients with antiSCL70 presented ILD in 50% of the sick patients and, besides, pulmonary involvement was observed in 75% of them in the first 5...
Inglés: Systemic sclerosis is a chronic autoimmune, multiorganic, rare disease, characterized by inflammation and mainly cutaneous fibrosis, associated with endothelial damage and inflammatory affection of blood vessels, osteomuscular tissues and internal organs. In addition an incidence of 19 cases per every 1,000,000 inhabitants is estimated in the United States with 19 cases per 1,000,000 inhabitants / year and its prevalence is up to 242 cases per 1,000,000 adults. According to the extent of skin involvement, SSc can be classified as diffuse (DSSc) or limited SSc (LSSc). The first is characterized by the generalized hardening of the skin, with frequent involvement of internal organs and the presence of antitopoisomerase I (Scl- 70) antibodies, which makes it the clinical presentation with the worst prognosis. SScL is characterized by toughening of the skin at distal level of extremities, such as face and neck, less involvement of damage in internal organs and presence of anticentromere antibodies with better prognosis compared with DSSc. Interstitial lung involvement can be observed in up to 75% of patients with systemic sclerosis and it's known to be the main cause of death. Therefore, it is necessary to describe the characteristics of our affected population. In order to diagnose interstitial lung disease, it is very important to have clinical data, the profile of antibodies associated with SSc, images obtained mainly by HRCT of thorax, and spirometric data for instance the decrease in FVC and DLCO. Data of 109 patients with systemic sclerosis has been analysed with an average age of 48 years. The 85.33% of the patients presented LSSc and the other 14.67% was associated with SScD. ILD was determined in 24% of the patients, who presented an ACA and antiSCL 70 antibody profile of 69.23% and 30.77%, respectively. Patients with antiSCL70 presented ILD in 50% of the sick patients and, besides, pulmonary involvement was observed in 75% of them in the first 5...
Descripción
Tesis (especialista en reumatología)--Universidad de Costa Rica. Sistema de Estudios de Posgrado. Programa de Estudios de Porgrado en Reumatología, 2019
Palabras clave
ENFERMEDADES INTERSTICIALES DE LOS PULMONES - CASOS, HISTORIAS CLÍNICAS, ESTADÍSTICAS, ETC. - COSTA RICA, ENFERMEDADES INTERSTICIALES DE LOS PULMONES - PACIENTES - COSTA RICA, ESCLERODERMIA SISTEMICA - PACIENTES - COSTA RICA, Hospital Dr. Max Peralta Jiménez (Costa Rica). Servicio de Reumatología, Hospital San Rafael de Alajuela (Costa Rica). Servicio de Reumatología