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Diffuse cystic lung disease in sickle cell anaemia: a series of 22 cases and a case–control study
  1. Fatma Kort1,
  2. Anoosha Habibi2,3,
  3. Francois Lionnet4,
  4. Marie-France Carette5,
  5. Antoine Parrot1,
  6. Laurent Savale6,
  7. Hilario Nunes7,
  8. Bernard Maitre8,9,
  9. Frederic Schlemmer10,11,
  10. Jean-Marc Naccache1,12,13
  1. 1 Service de Pneumologie, Assistance Publique-Hôpitaux de Paris, Hôpital Tenon, Paris, France
  2. 2 French Sickle Cell Referral Center, Assistance Publique-Hôpitaux de Paris, Hôpital Henri Mondor, Creteil, France
  3. 3 INSERM 955, Université Paris Est-Créteil, laboratoire d’excellence GR-Ex, Creteil, France
  4. 4 Service de Médecine Interne, Assistance Publique-Hôpitaux de Paris, Hôpital Tenon, Paris, France
  5. 5 Service de Radiologie, Assistance Publique-Hôpitaux de Paris, Hôpital Tenon, Paris, France
  6. 6 Service de Pneumologie, Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
  7. 7 Service de Pneumologie, Assistance Publique Hôpitaux de Paris, Hôpital Avicenne, Bobigny, France
  8. 8 Service de Pneumologie et de Pathologie Professionnelle, Centre Hospitalier Intercommunal de Créteil, Creteil, France
  9. 9 DHU A-TVB, Université Paris Est-Créteil, Creteil, France
  10. 10 Unité de Pneumologie, Assistance Publique-Hôpitaux de Paris, Hôpital Henri Mondor, Creteil, France
  11. 11 INSERM 955, Université Paris Est-Créteil, Creteil, France
  12. 12 Service de Pneumologie, Groupe Hospitalier Paris Saint Joseph, Paris, France
  13. 13 Service de Pneumologie, Hôpital Foch, Suresnes, France
  1. Correspondence to Dr Jean-Marc Naccache, Groupe hospitalier Paris Saint-Joseph, Paris, Île-de-France, France; jmnaccache{at}ghpsj.fr

Abstract

Chronic interstitial lung abnormalities have been described in sickle cell disease (SCD) and attributed to repetitive episode of acute chest syndrome. We report a series of 22 cases of diffuse cystic lung disease in SCD with a case–control study to hunt for mechanism. On pathological analysis of a surgical lung biopsy of the index case, the bronchioles had the appearance of constrictive bronchiolitis. Pulmonary function test results revealed lower forced expiratory flow from 25% to 75% of vital capacity in cases versus controls. These findings suggest a bronchiolar mechanism that was not associated with more acute chest syndrome.

  • rare lung diseases
  • imaging/CT MRI etc
  • interstitial fibrosis
  • systemic disease and lungs

https://doi.org/10.1136/thoraxjnl-2020-216826

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    Footnotes

    • Twitter @jm_nac

    • Contributors Substantial contributions to the conception and design of the work, planning and conducting the work, and analysis of the data: FK, AH, FL and J-MN. Acquisition and interpretation of data for the work; revising critically the manuscript for important intellectual content; final approval of the version submitted for publication; and agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved: all authors. Drafting of the manuscript: FK and J-MN.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.

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