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- Alberto Casey, Juan I Enghelmayer, Cora G Legarreta, Ana María Berón, María Marta Perín, and Diana Dubinsky.
- División de Neumonología, Hospital de Clínicas, Universidad de Buenos Aires, Buenos Aires, Argentina.
- Med Clin (Barc). 2024 Apr 12; 162 (7): 350353350-353.
IntroductionShrinking 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.Copyright © 2023 Elsevier España, S.L.U. All rights reserved.
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