Síndrome del pulmón encogido asociado a lupus eritematoso sistémico: estudio de 9 pacientes / Shrinking lung syndrome in systemic lupus erythematosus: A study of 9 patients

Introducción: El síndrome de pulmón encogido (SPE) es una manifestación rara del lupus eritematoso sistémico. Nuestro objetivo fue describir las características clínicas, radiológicas y funcionales de una cohorte con SPE y su evolución en el tiempo.MétodosEstudio retrospectivo entre 2009 y 2018. Se recogieron datos demográficos, clínicos, funcionales, radiológicos y de tratamiento.ResultadosDe un total de 225 pacientes, 11 presentaron SPE (prevalencia del 4,8%). Dos fueron excluidos. La edad media fue 39,33±16 años, 6 eran mujeres. Los síntomas principales fueron la disnea y el dolor pleurítico. La capacidad vital forzada media fue del 49%, la capacidad pulmonar total del 60%, la capacidad de difusión de monóxido de carbono del 66%, el factor de transferencia para el monóxido de carbono del 128%, la presión inspiratoria máxima del 66% y la presión espiratoria máxima del 82%. Todos los pacientes recibieron corticosteroides. Después de una mediana de seguimiento de 19 meses, 4 casos presentaron mejoría y 4 estabilización.ConclusionesEl SPE debe tenerse presente en todo paciente lúpico con disnea de causa no evidente. Si bien suele evolucionar con mejoría, la mayoría queda con deterioro persistente a pesar del tratamiento. (AU)

Introduction: Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus. Our aim was to describe the clinical, radiological, and functional characteristics of a cohort with SLS and its evolution over time.MethodsA retrospective study was conducted between 2009 and 2018. Demographic, clinical, functional, radiological, and treatment data were collected.ResultsOut of a total of 225 patients, 11 presented with SLS (prevalence of 4.8%). Two patients were excluded. The mean age was 39.33±16 years, and 6 were female. The main symptoms were dyspnea and pleuritic pain. The mean forced vital capacity was 49%, total lung capacity was 60%, carbon monoxide diffusing capacity was 66%, carbon monoxide transference factor was 128%, maximal inspiratory pressure was 66%, and maximal expiratory pressure was 82%. All patients received corticosteroids. After a median follow-up of 19 months, 4 cases showed improvement, and 4 cases remained stable.ConclusionsSLS should be considered in every lupus patient with unexplained dyspnea. Although it often shows improvement, many cases experience persistent deterioration despite treatment. (AU)

[Shrinking lung syndrome in systemic lupus erythematosus: A study of 9 patients]. / Síndrome del pulmón encogido asociado a lupus eritematoso sistémico: estudio de 9 pacientes.

INTRODUCTION: Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus. Our aim was to describe the clinical, radiological, and functional characteristics of a cohort with SLS and its evolution over time. METHODS: A retrospective study was conducted between 2009 and 2018. Demographic, clinical, functional, radiological, and treatment data were collected. RESULTS: Out of a total of 225 patients, 11 presented with SLS (prevalence of 4.8%). Two patients were excluded. The mean age was 39.33±16 years, and 6 were female. The main symptoms were dyspnea and pleuritic pain. The mean forced vital capacity was 49%, total lung capacity was 60%, carbon monoxide diffusing capacity was 66%, carbon monoxide transference factor was 128%, maximal inspiratory pressure was 66%, and maximal expiratory pressure was 82%. All patients received corticosteroids. After a median follow-up of 19 months, 4 cases showed improvement, and 4 cases remained stable. CONCLUSIONS: SLS should be considered in every lupus patient with unexplained dyspnea. Although it often shows improvement, many cases experience persistent deterioration despite treatment.