Management of acute chest syndrome in patients with sickle cell disease: a systematic review of randomized clinical trials

ABSTRACT

Introduction

Acute chest syndrome (ACS) accounts for the highest mortality in Sickle cell disease patients. Early diagnosis and timely management of ACS results in better outcomes. However, the effectiveness of most treatment modalities for ACS management has not been established.

Areas Covered

To review the treatment modalities management protocols and highlight the effectiveness of each option a literature search was done. Randomized controlled trials that assessed the efficacy of different treatment modalities in ACS management in SCD patients were chosen and reviewed.

Expert opinion

11 randomized controlled trials were found that evaluated the efficacy of incentive spirometry, positive expiratory pressure device, intravenous dexamethasone, oral vs. intravenous morphine, inhaled nitric oxide, unfractionated heparin, and blood transfusion in the prevention or treatment of ACS. Although there are guidelines for ACS treatment, the available evidence is very limited to delineating the effectiveness of various interventions in ACS management. More high-quality studies and trials with a larger patient population can benefit this area to support the recommendations with stronger evidence.

KEYWORDS:

• Acute chest syndrome (ACS)
• dexamethasone
• ECMO (extracorporeal membrane oxygenation)
• inhaled nitric oxide
• incentive spirometry
• morphine
• sickle cell disease (SCD)

Availability of data and materials

Data is available in excel files, patient identifying information was removed at all stages in all the studies included.

Article highlights

• Acute chest syndrome (ACS) is the leading cause of mortality and morbidity in Sickle cell disease (SCD) patients.

• ACS is defined as a new onset fever and/or respiratory symptoms accompanied by a new pulmonary infiltrate on the chest X-ray.

• Overall, there is a scarcity of trials to prove the utility of the treatment options used for ACS management. The main reason behind this lies in the small patient population and most of the trials being terminated due to patient dropouts.

• The current standard of care for ACS used in hospital settings is broadly comprised of (A) supportive care (oxygen supplementation, incentive spirometry, mechanical ventilation, hydration), (B) transfusion therapy (simple transfusion, exchange transfusion chronic, transfusion/hypertransfusion), and (C) pharmacotherapy (analgesics, antibiotics/antivirals, corticosteroids, bronchodilators, inhaled NO, anticoagulants, hydroxyurea).

• Upon literature review, 11 randomized controlled trials were found that evaluated the efficacy of incentive spirometry, positive expiratory pressure device, intravenous dexamethasone, oral vs. intravenous morphine, inhaled nitric oxide, unfractionated heparin, and blood transfusion in the prevention or treatment of ACS.

• To our knowledge, there are no randomized controlled trials completed to prove the efficacy of antibiotics, intravenous fluid hydration, extra-corporeal membrane oxygenation (ECMO), and bronchodilators for the treatment of ACS. They have mainly been used based on widely accepted expert opinion.

Declaration of interests

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Ethics approval and consent to participate

The data extracted and manuscript were reviewed with Research Department and Ethics Committee. No experimental intervention was performed, and any specification of guidelines, legislations, or permissions were not required.

Supplementary material

Supplemental data for this article can be accessed online at https://doi.org/10.1080/17474086.2022.2085089

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.