Pulmonary complications of sickle cell disease

Abstract

Sickle cell disease (SCD) is a common monogenetic disorder with high associated morbidity and mortality. The pulmonary complications of SCD are of particular importance, as acute chest syndrome and pulmonary hypertension have the highest associated mortality rates within this population. This article reviews the pathophysiology, diagnosis, and treatment of clinically significant pulmonary manifestations of SCD, including acute chest syndrome, asthma, and pulmonary hypertension in adult and pediatric patients. Clinicians should be vigilant in screening and treating such comorbidities to improve patient outcomes.

Figure 1.

Vicious cycle of vasoocclusive crisis and acute chest syndrome. Modified by permission from Reference .

Figure 2.

Radiographic evidence of pulmonary fibrosis related to multiple episodes of acute chest syndrome in two patients with sickle cell disease. Note the subpleural honeycombing fibrosis denoted by arrows.

Figure 3.

Radiographic findings of pulmonary hypertension in an 18-year-old patient with sickle cell disease. (A) Note the central pulmonary artery (PA) is enlarged and significantly larger than the adjacent ascending aorta (AA), indicating a PA/AA ratio greater than 1. (B, C) Relative increase in segmental artery size relative to adjacent bronchus (arrows) as well as loss of peripheral vascularity. (C) Note right ventricle (RV) is hypertrophied and dilated with evidence of right atrial (RA) dilation. All images illustrate the finding of mosaic perfusion pattern of parenchymal attenuation.

Figure 4.

Screening and treatment approach for adult patients with sickle cell disease (SCD). The role of screening Doppler echocardiography, plasma N-terminal pro-brain natriuretic peptide (NT-proBNP), and 6-minute walk, with confirmation by right heart catheterization, is outlined. The prevalence and hemodynamic definitions of cardiopulmonary abnormalities found on screening are summarized. mPAP = mean pulmonary artery pressure; PAH = pulmonary arterial hypertension; PCWP = pulmonary capillary wedge pressure; PH = pulmonary hypertension; TRV = tricuspid regurgitant jet velocity.