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Case Report: Diaphragm Ultrasound Reveals Shrinking Lung Syndrome

Mery Deeb, MD, Taro Minami, MD, Michael Stanchina, MD, Elias Jabbour, MD, & Jan Karczewski, MD  |  Issue: July 2024  |  

In Sum

Shrinking lung syndrome continues to be under-recognized overall, although awareness of the condition has increased over the past few years. Contrary to popular belief, shrinking lung syndrome could be an early feature of SLE. We should keep a high index of suspicion among patients with connective tissue disease who present with dyspnea, pleuritic chest pain and elevation of the diaphragm.

Although PFTs and chest CT remain essential to the diagnosis, diaphragm ultrasound can help confirm diaphragmatic dysfunction and supports a shrinking lung syndrome diagnosis, particularly in challenging cases. Given the potential for chronic restrictive lung physiology, early diagnosis may minimize long-term respiratory dysfunction.

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Mery Deeb, MD, is a resident in internal medicine at the Department of Medicine, Brown University, Providence, R.I., and Kent Hospital, Warwick, R.I.

Taro Minami, MD, is with the Department of Medicine, Division of Pulmonary, Critical Care, and Sleep Medicine, The Warren Alpert Medical School of Brown University, Providence, R.I.

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Michael Stanchina, MD, is with the Department of Medicine, Division of Pulmonary, Critical Care, and Sleep Medicine, The Warren Alpert Medical School of Brown University, Providence, R.I., and the Division of Pulmonary/Critical Care Medicine, Brigham and Women’s Hospital, Boston, and Care New England Hospitals, Rhode Island.

Elias Jabbour, MD, is with the Division of Pulmonary/Critical Care Medicine, Brigham and Women’s Hospital, Boston, and Care New England Hospitals, Rhode Island.

Jan Karczewski, MD, is with the Division of Rheumatology, Kent Hospital, Warwick, R.I.

Disclosures

Dr. Minami served as a consultant for Fujifilm (until 2023) and AA Health Dynamics (until January 2024) to teach point-of-care ultrasound in Kenya, a project funded by Ministry of Economy, Trade and Industry.

References

  1. Hoffbrand BI, Beck ER. ‘Unexplained’ dyspnoea and shrinking lungs in systemic lupus erythematosus. Br Med J. 1965 May 15;1(5445):1273–1277.
  2. Hoffman BJ. Sensitivity to sulfadiazine resembling acute disseminated lupus erythematosus. Arch Dermatol Syphilol. 1945;51(3):190–192.
  3. Minami T, Manzoor K, McCool FD. Assessing diaphragm function in chest wall and neuromuscular diseases. Clin Chest Med. 2018 Jun;39(2):335–344.
  4. McCool FD, Tzelepis GE. Dysfunction of the diaphragm. N Engl J Med. 2012 Mar 8;366(10):932–942.
  5. Yamada T, Minami T, Yoshino S, et al. Diaphragm ultrasonography: Reference values and influencing factors for thickness, thickening fraction, and excursion in the seated position. Lung. 2024 Feb;202(1):83–90.
  6. Borrell H, Narváez J, Alegre JJ, et al. Shrinking lung syndrome in systemic lupus erythematosus: A case series and review of the literature. Medicine (Baltimore). 2016 Aug;95(33):e4626.
  7. Henderson LA, Loring SH, Gill RR, et al. Shrinking lung syndrome as a manifestation of pleuritis: A new model based on pulmonary physiological studies. J Rheumatol. 2013 Mar;40(3):273–281.
  8. Hawkins P, Davison AG, Dasgupta B, et al. Diaphragm strength in acute systemic lupus erythematosus in a patient with paradoxical abdominal motion and reduced lung volumes. Thorax. 2001 Apr;56(4):329–330.
  9. Laroche CM, Mulvey DA, Hawkins PN, et al. Diaphragm strength in the shrinking lung syndrome of systemic lupus erythematosus. Q J Med. 1989 May;71(265):429–439.
  10. DeCoste C, Mateos-Corral D, Lang B. Shrinking lung syndrome treated with rituximab in pediatric systemic lupus erythematosus: A case report and review of the literature. Pediatr Rheumatol Online J. 2021 Jan 6;19(1):7.
  11. Goswami RP, Mondal S, Lahiri D, et al. Shrinking lung syndrome in systemic lupus erythematosus successfully treated with rituximab. QJM. 2016 Sep;109(9):617–618.
  12. Langenskiöld E, Bonetti A, Fitting JW, et al. Shrinking lung syndrome successfully treated with rituximab and cyclophosphamide. Respiration. 2012;84(2):144–149.
  13. Peñacoba Toribio P, Córica Albani ME, Mayos Pérez M, Rodríguez de la Serna A. Rituximab in the treatment of shrinking lung syndrome in systemic lupus erythematosus. Reumatol Clin. 2014 Sep-Oct;10(5):325–327.

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