Chest
Recent Advances in Chest MedicineChronic Pulmonary Complications of Sickle Cell Disease
Section snippets
Definitions
Pulmonary hypertension (PH), defined hemodynamically as a mean pulmonary arterial pressure ≥ 25 mm Hg at rest, occurs in 6% to 10.4% of adults with SCD.3, 4, 5, 6 Right-sided heart catheterization is the gold standard for the diagnosis of PH3 and assessment of prognosis. The hemodynamics of PH in patients with SCD are heterogeneous (Table 1). PH may be precapillary (pulmonary arterial hypertension [PAH]), postcapillary (pulmonary venous hypertension), or have features of both.7 Clinical
Venous Thromboembolism in SCD
VTE (deep vein thrombosis and pulmonary embolism [PE]) affects about 25% of adults with SCD and is a risk factor for death.30, 31 Despite the known hypercoagulability of SCD, VTE is often overlooked as a major complication of SCD,30 in part because in situ microvascular thrombosis occurs in these patients and large-vessel thromboses have been recognized only with diagnostic advances.
The cause of increased VTE risk in SCD is multifactorial. Patients with SCD have traditional VTE risk factors and
Sleep-Disordered Breathing and Nocturnal Hypoxemia
SDB, particularly OSA and intermittent oxyhemoglobin desaturation, are potential contributors to cardiopulmonary compromise in SCD, possibly via increasing vaso-occlusion.47, 48 Despite this, SDB is underappreciated and, consequently, often undertreated; this is due, in part, to a lack of understanding of its prevalence and natural history in SCD.
Much of what is known about SDB in SCD stems from retrospective cohort studies in which up to 79% of symptomatic children and adolescents were
Asthma and Recurrent Wheezing
In the United States, African Americans and people of mixed race have a higher prevalence of asthma than do white individuals with an increased mortality rate.62 One of the difficulties in diagnosing asthma in SCD stems from the overlap of clinical features that may be due solely to SCD63, 64 and those of asthma in the non-SCD population (Table 5). Koumbourlis et al reported that 35% of 5- to 18-year-old patients with SCD had lower airway obstruction.64 In addition, up to 77% of children and
Adults
Pulmonary function testing (PFT) often produces abnormal results in adults with SCD.76, 77, 78 A cross-sectional analysis of 310 HbSS adults demonstrated abnormal pulmonary function in 90%, predominantly restrictive physiology (mildly decreased total lung capacity and diffusion capacity of the lung for carbon monoxide [Dlco]).77 Longitudinal studies of adults with SCD demonstrate an average decline in forced expiratory volume in 1 second of 49 mL/y (unrelated to smoking status) compared with 20
Summary and Future Directions
Pulmonary complications of SCD represent a diverse group of diseases affecting the large and small airways, parenchyma, and vasculature. Although pulmonary disease clearly has an adverse impact on outcomes in SCD, much needs to be learned about the natural history of these conditions across the life span of patients with SCD to better understand the potential impacts of screening and prevention. Moreover, clinical trials that target this population specifically are needed, because it is
Acknowledgment
Financial/nonfinancial disclosures: The authors have reported to CHEST the following: E. S. K. has received grant support from Actelion Pharmaceuticals and Pfizer. None declared (A. M.).
Role of sponsors: The sponsor had no role in the design of the study, the collection and analysis of the data, or the preparation of the manuscript.
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FUNDING/SUPPORT: Dr Mehari is supported by National Heart, Lung, and Blood Institute of the National Institutes of Health under Award Number P50HL118006 and Department of Medicine Academic Enrichment Fund.