Vol. 18 No. 3 (2024): Revista Reumatología al Día
Articles

Shrinking lung syndrome in systemic lupus erythematosus: A rare entity. Report of 6 cases.

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  • J. J. Astudillo Andrade,
  • M. Micelli,
  • V. Cosentino,
  • E. Kerzberg
J. J. Astudillo Andrade
Servicio de Reumatología y Colagenopatías. Hospital J. M. Ramos Mejía; CABA, Argentina.
DOI: https://doi.org/10.62484/rad.v18i3.9257

Published 2024-12-24

Keywords

  • shrinking lung syndrome,
  • systemic lupus erythematosus,
  • unexplained dyspnea,
  • restrictive ventilatory pattern

How to Cite

Astudillo Andrade, J. J., Micelli, M., Cosentino, V., & Kerzberg, E. (2024). Shrinking lung syndrome in systemic lupus erythematosus: A rare entity. Report of 6 cases. Reumatología Al Día, 18(3), 9. https://doi.org/10.62484/rad.v18i3.9257

Copyright (c) 2024 Reumatología al Día

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Abstract

One of the rare respiratory manifestations in SLE is the shrinking lung syndrome, clinically characterized by unexplained dyspnea, pleuritic pain, and a characteristic triad: lung volume reduction, diaphragmatic elevation, and restrictive ventilatory defect.

Objective: To describe the clinical characteristics of a series of 6 patients diagnosed with SLE-associated PES and their relationship with activity and damage.

Method: Retrospective study, with data obtained from medical records. Demographic, clinical, serological, and radiological data were collected at diagnosis and at 6 months.

Results: Of 220 patients diagnosed with SLE, 6 met all criteria for PES, calculating a prevalence of 2.7%; 4 were women, with a mean age of 31.3 (+-9.5) years; the time between the onset of SLE and PES was 72 (+-67.7) months. At diagnosis, 100% had dyspnea and orthopnea; The most frequent concomitant non-respiratory manifestations were: malar rash (83.3%), arthritis, arthralgias and lymphopenia (66.6%). All patients (100%) were interpreted as active to present a SLEDAI 2K >4. At 6 months after diagnosis, 66.7% were inactive, but the damage caused by the disease according to the SLICC/ACR SDI increased in 100% of the patients.

Conclusions: In the presence of unexplained dyspnea, we must think about rare pathologies such as EPS. Relating its presence to the activity of the disease is important and due to the favorable response to corticosteroids, its good evolution and prognosis, it is necessary to always have the suspicion of this pathology.

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