Shrinking lung syndrome in systemic lupus erythematosus: A study of 9 patients

• Published in: Medicina clinica Vol. 162; no. 7; p. 350
• Main Authors: Casey, Alberto, Enghelmayer, Juan I, Legarreta, Cora G, Berón, Ana María, Perín, María Marta, Dubinsky, Diana
• Format: Journal Article
• Language: English
• Published: Spain 12-04-2024
• Subjects: Adult; Carbon Monoxide - therapeutic use; Digestive System Diseases; Dyspnea - etiology; Female; Humans; Lung - diagnostic imaging; Lung Diseases - diagnosis; Lupus Erythematosus, Systemic - diagnosis; Male; Middle Aged; Muscular Diseases; Retrospective Studies; Syndrome; Young Adult; Síndrome de pulmón encogido; Systemic lupus erythematosus; Restrictive lung disease; Shrinking lung syndrome; Enfermedad pulmonar restrictiva; Lupus eritematoso sistémico
• ISSN: 1578-8989
• DOI: 10.1016/j.medcli.2023.10.023

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.
A retrospective study was conducted between 2009 and 2018. Demographic, clinical, functional, radiological, and treatment data were collected.
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.
SLS should be considered in every lupus patient with unexplained dyspnea. Although it often shows improvement, many cases experience persistent deterioration despite treatment.