Pulmonary function in sickle cell disease with or without acute chest syndrome

Abstract

Recurrent acute chest syndrome (ACS) has been suggested as a risk factor for chronic lung dysfunction in sickle cell disease. To investigate this hypothesis, lung function tests were performed in 49 sickle cell disease outpatients whose condition was stable, including 23 patients with a history of two to four episodes of ACS (ACS+) and 26 with no history of ACS (ACS-). The two groups were comparable regarding the sex ratio, body mass index, smoking history, physical characteristics, clinical history and usual lung function tests. Respiratory resistance (Rrs), measured using the forced oscillation technique, increased with the number of ACS episodes (r=0.55, p<0.0001) and a significant relationship was observed between Rrs as an independent variable and the expiratory flow rates at 25, 50 and 25-75% of the forced vital capacity as explanatory variables (r= 0.36, p<0.02; r=0.35, p<0.02; and r=0.4, p<0.006, respectively), with higher Rrs being associated with lower expiratory flow rates. The transfer factor (TL,CO) and transfer coefficient (KCO) for CO were significantly higher in the ACS+ group than in the ACS-group (TL,CO=84+/-4 versus 71+/-3%, p<0.004 and KCO=102+/-5 versus 90+/-3%, p<0.05, respectively). The data demonstrate that obstructive lung dysfunction is fairly common in sickle cell disease and suggest that recurrent acute chest syndrome may contribute specific obstructive defects. The increase in respiratory resistance associated with acute chest syndrome was accompanied by an increase in diffusion capacity, suggesting that it may have been related to an increase in lung blood volume.

Progression and Prognostic Indicators of Bronchial Disease in Children with Sickle Cell Disease

“…Earlier studies emphasize chronic vascular injury and parenchymal disease as an important baseline factor in determining the progression of pulmonary dysfunction in children with SCD [23][24][25][26]. However, our study demonstrates earlier signs of bronchial disease and airway obstruction as reflected by declining FEV1% of predicted has a stronger correlation with disease progression.…”

The forced oscillation technique in clinical practice: methodology, recommendations and future developments

“…In sickle cell disease, an increase in Rrs is correlated with the number of acute chest syndrome episodes, which demonstrates that obstructive lung dysfunction is fairly common in this type of disease [83].…”

Abnormal Pulmonary Function in Adults with Sickle Cell Anemia

“…Studies of lung function to date in this population have been of modest size, often involving fewer than 50 patients (4,(6)(7)(8)(9)(10)(11) and largely inconclusive. Their results have yielded a spectrum of abnormalities, including restrictive lung disease, abnormal diffusion capacity for carbon monoxide (Dl CO ), obstructive disease, and hypoxemia (4,6,7,9,12). No definitive profile for pulmonary function in sickle cell disease has emerged.…”

“…The few published studies suggest that obstructive lung disease, in some cases, plays a role in the pathogenesis of ACS (7,13,15). Moreover, obstructive lung disease could be a long-term sequela of recurrent episodes of ACS (6,9). Larger scale studies are necessary to elucidate more clearly the interaction between lung function and ACS.…”