Síndrome del pulmón encogido asociado a lupus eritematoso sistémico / Shrinking lung syndrome associated with systemic lupus erythematosus
Medicina (B.Aires)
; Medicina (B.Aires);84(4): 635-640, ago. 2024.
Article
em Es
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LILACS-Express
| LILACS
| ID: biblio-1575257
Biblioteca responsável:
AR1.1
RESUMEN
Resumen Introducción:
El síndrome de pulmón encogido (SPE) es una manifestación rara y poco conocida del lupus eritematoso sistémico (LES). El objetivo es describir las características demográficas, manifestaciones clínicas, funcionales, imagenológicas y tratamiento recibido en una cohorte de pacientes diagnosticados con SPE.Métodos:
Se examinaron las historias clínicas de una cohorte de pacientes con LES seguidos prospectivamente desde 2007 al 2023 por los Servicios de Reumatología y Neumotisiología.Resultados:
De 107 pacientes diagnosticados con LES, se hallaron 11 pacientes con SPE (10.3%), 81.8% fueron mujeres y 18.2 % varones; edad (mediana) 30 años (RIQ 25-75% = 25.5-41.5). Presentaron disnea 72%, dolor pleu rítico 36%, y asintomáticos 27.3% al momento del diag nóstico. Funcionalmente presentaron FEV1 / FVC = 86% mediana, (RIQ = 82-90), FVC mediana 61% (RIQ= 38.5-71), DLCO mediana 65% (RIQ = 48-69) y DLCO/VA mediana 95% (RIQ = 89-106). Se observó en la tomografía de tórax de alta resolución (TACAR) atelectasias (54.5%), elevación del diafragma (27.3%), normalidad (27.3%) y derrame pleural (9%). Recibieron tratamiento con corticoides sistémicos (100%), asociados a otros inmunosupresores en 72.7% metotrexato (36%), micofenolato (27.3%), hi droxicloroquina (27.3%) y azatioprina (18.2%).Conclusión:
La prevalencia de SPE fue 10.3%, más alta que lo reportado en otras series de casos de LES. La dis nea fue el síntoma principal de consulta. Los pacientes presentaron funcionalmente sospecha de restricción moderada y leve caída de la DLCO. La atelectasia fue el signo más frecuente en la TACAR. El tratamiento in munosupresor fue indicado en todos los pacientes con corticoides sistémicos asociados usualmente a otros inmunosupresores.ABSTRACT
Abstract Introduction:
Shrinking lung syndrome (SLS) is a rare and less-known manifestation of systemic lupus erythematosus (SLE). The aim of this study is to describe the demographic, clinical, functional, imaging charac teristics, and treatment received in a cohort of patients diagnosed with SLS.Methods:
Clinical records were reviewed retrospec tively in a cohort of patient with SLE and SLS followed-up prospectively between 2007 to 2023 in the Rheuma tology and Neumonology Units.Results:
Eleven SLS patients (10.3%) of 107 SLE pa tients were diagnosed. They were 81.8% female and 18.2% male. Median age was 30 years old (RIQ = 25-75% [25.5-41.5]). Clinical symptoms were dyspnea (72%), pleuritic pain (36%), but 27.3% of patients were asymp tomatic at diagnosis. Respiratory functional evaluation was, median FEV1 / FVC 86% (RIQ = 82-90), median FVC 61% (RIQ = 38.5-71), median DLCO 65% (RIQ = 48-69) and median DLCO/VA 95% (RIQ = 89-106). High-resolution computed tomography of thorax (HRCT) showed atel ectasis (54.5%), diafragmatic elevation (27.3%), normal (27.3%) and pleural effusion (9%). Patients were treated with systemic corticosteroids (100%); in 72.7% of them, associated to other immunosupressives methotrex ate (36%), mycophenolate (27.3%), hydroxychloroquine (27.3%) and azatioprine (18.2%).Conclusion:
SLS prevalence was 10.3%, higher than reported in other studies of SLE. Dyspnea was the most clinical symptom. Suspicion of moderate restriction were determined with mild deterioration of DLCO. At electasis was the most frequent HCRT sign. Immunosu pressive treatment was prescribed in all patients with systemic corticosteroids associated to other drugs.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
LILACS
Idioma:
Es
Revista:
Medicina (B.Aires)
Assunto da revista:
MEDICINA
Ano de publicação:
2024
Tipo de documento:
Article
País de afiliação:
Argentina
País de publicação:
Argentina