open access

Vol 89, No 2 (2021)
Review paper
Published online: 2021-04-21
Submitted: 2020-10-26
Accepted: 2020-11-30
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Pulmonary complications of sickle cell disease: a narrative clinical review

Amina Pervaiz, Firas El-Baba, Kunwardeep Dhillon, Asil Daoud, Ayman Soubani
DOI: 10.5603/ARM.a2021.0011
·
Pubmed: 33881157
·
Adv Respir Med 2021;89(2):173-187.

open access

Vol 89, No 2 (2021)
REVIEWS
Published online: 2021-04-21
Submitted: 2020-10-26
Accepted: 2020-11-30

Abstract

Sickle cell disease (SCD) is associated with vaso-occlusive episodes that affect different organs. Pulmonary involvement is a major cause of morbidity and mortality in this patient population.
We performed a literature search in the PubMed database for articles addressing SCD and pulmonary diseases. Acute chest syndrome is defined as a new radiodensity on chest radiograph imaging with a history consistent of the disease. Management includes broad spectrum antibiotics, pain control, and blood transfusions. Microvasculature infarcts lead to functional asplenia, which in turn increases the risk of being infected with encapsulated organisms. Universal vaccinations and antibiotic prophylaxis play a significant role in decreasing mortality from pulmonary infections. Venous thromboembolism in patients with SCD should be treated in the same manner as in the general population. Pulmonary hypertension in patients with SCD also increases mortality. The American Thoracic Society treatment modalities are based on the underlying etiology which is either directed at treating SCD itself, using vasodilator medications if the patient is in group 1, or using long-term anticoagulation if the patient is group 4 (in terms of etiology). Patients with SCD are more likely to suffer from asthma in comparison to controls. Sleep disorders of breathing should be considered in patients with unexplained nocturnal and daytime hypoxemia, or recurrent vaso-occlusive events. Lastly, the utility of pulmonary function tests still needs to be established.

Abstract

Sickle cell disease (SCD) is associated with vaso-occlusive episodes that affect different organs. Pulmonary involvement is a major cause of morbidity and mortality in this patient population.
We performed a literature search in the PubMed database for articles addressing SCD and pulmonary diseases. Acute chest syndrome is defined as a new radiodensity on chest radiograph imaging with a history consistent of the disease. Management includes broad spectrum antibiotics, pain control, and blood transfusions. Microvasculature infarcts lead to functional asplenia, which in turn increases the risk of being infected with encapsulated organisms. Universal vaccinations and antibiotic prophylaxis play a significant role in decreasing mortality from pulmonary infections. Venous thromboembolism in patients with SCD should be treated in the same manner as in the general population. Pulmonary hypertension in patients with SCD also increases mortality. The American Thoracic Society treatment modalities are based on the underlying etiology which is either directed at treating SCD itself, using vasodilator medications if the patient is in group 1, or using long-term anticoagulation if the patient is group 4 (in terms of etiology). Patients with SCD are more likely to suffer from asthma in comparison to controls. Sleep disorders of breathing should be considered in patients with unexplained nocturnal and daytime hypoxemia, or recurrent vaso-occlusive events. Lastly, the utility of pulmonary function tests still needs to be established.

Get Citation

Keywords

sickle cell disease; acute chest syndrome; pneumonia; venous thromboembolic disease; pulmonary hypertension

About this article
Title

Pulmonary complications of sickle cell disease: a narrative clinical review

Journal

Advances in Respiratory Medicine

Issue

Vol 89, No 2 (2021)

Article type

Review paper

Pages

173-187

Published online

2021-04-21

DOI

10.5603/ARM.a2021.0011

Pubmed

33881157

Bibliographic record

Adv Respir Med 2021;89(2):173-187.

Keywords

sickle cell disease
acute chest syndrome
pneumonia
venous thromboembolic disease
pulmonary hypertension

Authors

Amina Pervaiz
Firas El-Baba
Kunwardeep Dhillon
Asil Daoud
Ayman Soubani

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