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Inicio Medicina Clínica (English Edition) Shrinking lung syndrome in systemic lupus erythematosus: A study of 9 patients
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Vol. 162. Issue 7.
Pages 350-353 (April 2024)
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Vol. 162. Issue 7.
Pages 350-353 (April 2024)
Clinical report
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
Alberto Caseya, Juan I. Enghelmayera,
Corresponding author
jiedsn@gmail.com

Corresponding author.
, Cora G. Legarretaa, Ana María Berónb, María Marta Perína, Diana Dubinskyb
a División de Neumonología, Hospital de Clínicas, Universidad de Buenos Aires, Buenos Aires, Argentina
b División de Reumatología, Hospital de Clínicas, Universidad de Buenos Aires, Buenos Aires, Argentina
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Table 1. Clinical, radiological, functional, treatment and progress data.
Abstract
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 66.6% 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 for CO 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.

Keywords:
Shrinking lung syndrome
Systemic lupus erythematosus
Restrictive lung disease
Resumen
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 SLS y su evolución en el tiempo.

Métodos

Estudio retrospectivo entre 2009 y 2018. Se recogieron datos demográficos, clínicos, funcionales, radiológicos y de tratamiento.

Resultados

De un total de 225 pacientes, 11 presentaron SLS (prevalencia de 4.8%). Dos fueron excluidos. La edad media fue 39.33±16 años, 66.6% mujeres. Los síntomas principales fueron la disnea y el dolor pleurítico. La capacidad vital forzada media fue de 49%, la capacidad pulmonar total 60%, la capacidad de difusión de CO 66%, factor de transferencia para el CO 128%, presión inspiratoria máxima 66% y la presión espiratoria máxima 82%. Todos los pacientes recibieron corticosteroides. Después de una mediana de seguimiento de 19 meses, 4 casos presentaron mejoría y 4 estabilización.

Conclusiones

El SLS 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.

Palabras clave:
Síndrome de pulmón encogido
Lupus eritematoso sistémico
Enfermedad pulmonar restrictiva

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