Skip to content

ECAM

An overview of pulmonary manifestations in sickle cell disease

Lungs in sickle cell disease

Abstract

The increased survival of patients with sickle cell disease (SCD) into adulthood leads an increased incidence of multiorgan dysfunction. The lung is one of the most common organs that may be involved in SCD. The pulmonary manifestations of SCD are the leading cause of morbidity and mortality in these patients. Although pulmonary manifestations of SCD are very common, they remain underdiagnosed by clinicians. The main pulmonary manifestations of SCD are acute chest syndrome, chronic dyspnea, asthma, sleep-disordered breathing, acute and chronic venous thromboembolic disease, pulmonary hypertension, and pulmonary fibrosis. Herein, the current knowledge of the pathophysiology, diagnosis, and treatment of pulmonary manifestations of SCD are reviewed.

Keywords

sickle cell diseasepulmonary manifestationacute chest syndrome

Conclusion

Pulmonary complications are common causes of death in patients with SCD. The pulmonary manifestations of SCD are difficult to manage because they result from the complicated and complex pathophysiology of SCD. Considering that pulmonary manifestations are responsible for a big proportion of mortalities in SCD patients, clinicans should be aware of acute and chronic complications of SCD.

Declarations

Informed Consent

Informed consent was not required because this study was a review article and did not involve identifiable human data.

Conflict of Interest

The authors declare that there is no conflict of interest.

Funding

None.

References

  1. Embury SH, Vichinsky EP. Sickle cell disease. In: Hoffman R, Benz EJ Jr, Shattil SJ, et al, eds. Hematology: Basic principles and practice. 3rd ed. Churchill Livingstone; 2000:510-554.
  2. Ergul S, Brunson CY, Hutchinson J, et al. Vasoactive factors in sickle cell disease: in vitro evidence for endothelin-1–mediated vasoconstriction. Am J Hematol. 2004;76:245. doi:10.1002/ajh.20107
  3. Minter KR, Gladwin MT. Pulmonary complications of sickle cell anemia: a need for increased recognition, treatment, and research. Am J Respir Crit Care Med. 2001;164:2016. doi:10.1164/ajrccm.164.11.2104101
  4. Ocal N, Ocal R, Dogan D. An overview of eosinophilic lung diseases. J Ann Eur Med. 2016;4(1):12-15.
  5. Ocal N, Dogan D, Cicek AF, et al. Acute eosinophilic pneumonia with respiratory failure induced by synthetic cannabinoid inhalation. Balkan Med J. 2015. doi:10.5152/balkanmedj.2016.151145
  6. Ocal N, Dogan D, Ocal R, et al. Effects of radiological extent on neutrophil/lymphocyte ratio in pulmonary sarcoidosis. Eur Rev Med Pharmacol Sci. 2016;20(4):709-714.
  7. Castro O, Brambilla DJ, Thorington B, et al. The acute chest syndrome in sickle cell disease: incidence and risk factors. Blood. 1994;84:643-649. doi:10.1182/blood.v84.2.643.643
  8. Ocal N, Dogan D, Ozkisa T, Deniz O, Bilgic H. Septic pulmonary embolism: a case report. J Clin Anal Med. 2014;5(3):318-320.
  9. Klings ES, Wyszynski DF, Nolan VG, Steinberg MH. Abnormal pulmonary function in adults with sickle cell anemia. Am J Respir Crit Care Med. 2006;173:1264. doi:10.1164/rccm.200601-125oc
  10. Knight-Madden JM, Forrester TS, Lewis NA, Greenough A. Asthma in children with sickle cell disease and its association with acute chest syndrome. Thorax. 2005;60:206. doi:10.1136/thx.2004.029165
  11. Boyd JH, Macklin EA, Strunk RC, DeBaun MR. Asthma is associated with acute chest syndrome and pain in children with sickle cell anemia. Blood. 2006;108:2923-2927. doi:10.1182/blood-2006-01-011072
  12. Kaleyias J, Mostofi N, Grant M, et al. Severity of obstructive sleep apnea in children with sickle cell disease. J Pediatr Hematol Oncol. 2008;30:659. doi:10.1097/mph.0b013e31817eb7ef
  13. Rogers VE, Lewin DS, Winnie GB, Geiger-Brown J. Polysomnographic characteristics of a referred sample of children with sickle cell disease. J Clin Sleep Med. 2010;6:374. doi:10.5664/jcsm.27880
  14. Glassberg JA, Strunk R, DeBaun MR. Wheezing in children with sickle cell disease. Curr Opin Pediatr. 2014;26:9. doi:10.1097/mop.0000000000000045
  15. Naik RP, Streiff MB, Lanzkron S. Sickle cell disease and venous thromboembolism: what the anticoagulation expert needs to know. J Thromb Thrombolysis. 2013;35:352. doi:10.1007/s11239-013-0895-y
  16. Mekontso Dessap A, Deux JF, Abidi N, et al. Pulmonary artery thrombosis during acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med. 2011;184:1022. doi:10.1164/rccm.201105-0783oc
  17. Klings ES, Machado RF, Barst RJ, et al. An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. Am J Respir Crit Care Med. 2014;189:727. doi:10.1164/rccm.201401-0065st
  18. Haque AK, Gokhale S, Rampy BA, et al. Pulmonary hypertension in sickle cell hemoglobinopathy: a clinicopathologic study of 20 cases. Hum Pathol. 2002;33:1037. doi:10.1053/hupa.2002.128059
  19. Sachdev V, Machado RF, Shizukuda Y, et al. Diastolic dysfunction is an independent risk factor for death in patients with sickle cell disease. J Am Coll Cardiol. 2007;49(4):472-479. doi:10.1016/j.jacc.2006.09.038
  20. Dosunmu AO, Akinola RA, Onakoya JA, et al. Pattern of chronic lung lesions in adults with sickle cell disease in Lagos, Nigeria. Caspian J Intern Med. 2013;4:754.
  21. Koumbourlis AC. Lung function in sickle cell disease. Paediatr Respir Rev. 2014;15:33. doi:10.1016/j.prrv.2013.10.002

Additional Information

Publisher’s Note
Bayrakol MP remains neutral with regard to jurisdictional and institutional claims.

Rights and Permissions

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.[CC BY-NC 4.0] To view a copy of the license, visit https://creativecommons.org/licenses/by-nc/4.0/

About This Article

How to Cite This Article

Ramazan Öcal. An overview of pulmonary manifestations in sickle cell disease. Eu Clin Anal Med 2026;4(2):68. doi:10.4328/ECAM.84

Received:
23.03.2016
Accepted:
13.04.2016
Published Online:
01.05.2016
Printed:
01.05.2016