Asthma and the outcome of sickle cell disease. (2nd December 2018)

• Record Type: Journal Article
• Authors: Lunt, Alan; Sturrock, Sarah S.; Greenough, Anne
• Is Part Of: Expert opinion on orphan drugs. Volume 6:Number 12(2018)
• Page Start: 733
• Page End: 740
• Publication Date: 2018-12-02
• Subjects: Acute chest syndrome -- asthma -- sickle cell disease -- wheezing; Orphan drugs -- Periodicals; Rare diseases -- Periodicals; Chemotherapy -- Periodicals; 615.1
• DOI: 10.1080/21678707.2018.1547964
• Languages: English
• ISSNs: 2167-8707

Abstract

ABSTRACT: Introduction : Sickle cell disease (SCD) is one of the commonest monogenetic diseases. Worldwide, there are 300, 000 infants with SCD born every year. Affected patients can die prematurely in their fifth decade as they suffer multi-organ damage, including pulmonary complications. Areas covered : This review describes the pathophysiology of SCD and its pulmonary complications and discusses the relationship between asthma and the outcomes of SCD. Patients with SCD and asthma, but importantly recurrent episodes of wheeze (which may not be due to asthma) have an increased risk of acute chest syndrome (ACS), painful crisis, stroke, and all-cause mortality. In SCD, there is an increased pulmonary vascular volume, which is a response to their chronic anemia. Data from adult and pediatric cohorts show such hemodynamic changes result in airflow obstruction and hence wheezing. Expert commentary : The wheezing and lung function abnormalities which occur in SCD patients can be due to asthma, but in others reflects the vascular airway interaction occurring because of chronic anemia. It is important to differentiate whether a SCD patient is wheezing due to asthma or SCD per se as it will affect the choice of appropriate treatment.