Acute chest syndrome in sickle cell anaemia: higher serum levels of interleukin-8 and highly sensitive C-reactive proteins are associated with impaired lung function.

BACKGROUND

Sickle cell anaemia (SCA) is a chronic inflammatory disorder with multiple organ manifestations including acute and long-term pulmonary dysfunction.

AIMS/OBJECTIVES: To assess lung function of children with SCA and determine the possible role of acute chest syndrome (ACS), serum inflammatory cytokines, highly sensitive C-reactive protein (hs-CRP), leucocytes and 25-hydroxyvitamin D on the development of impaired lung function.

SUBJECTS AND METHODS

Lung function of 76 children with SCA was determined by spirometer and classified into normal or impaired. Sociodemographic, clinical, haematological, biochemical and immunological data of the two groups were compared by univariate and multivariate analyses.

RESULTS

Fifty (65.8%) patients had impaired lung function, comprising of 30.3%, 3.9% and 31.6% with restrictive, obstructive and mixed disease patterns, respectively. Children with previous ACS were 3.6 times more likely to have impaired lung function than those without ACS (82.1% vs 56.3%, p = 0.02, OR 3.6, 95% CI 1.2-10.8). Interleukin (IL)-8 and hs-CRP were significantly higher in patients with impaired lung function (p = 0.02 and <0.001, respectively). Using logistic regression, previous ACS (OR 5.8, 95% CI 1.1-5.8, p = 0.03) and higher serum IL-8 levels (OR 3.0, 95% CI 1.0-8.0, p = 0.02) independently predicted the presence of abnormal lung function.

CONCLUSIONS

Lung dysfunction, predominantly restrictive pattern, is common in SCA and is associated with previous ACS and alterations in immunological markers, especially serum IL-8 and hs-CRP.

ABBREVIATIONS

ACS: acute chest syndrome; CBT: chronic blood transfusion; ELISA: enzyme-linked immunosorbent assay; FEV: forced expiratory volume in 1 s; FVC: forced vital capacity; HPLC: high-density liquid chromatography; hs-CRP: highly sensitive C-reactive proteins; HU: hydroxyurea; IL: Interleukin; PEFR: peak expiratory flow rate; SEM: standard error of the mean; TLC: total lung capacity; 25-OHD: 25-hydroxyvitamin D; VOC: vaso-occlusive crisis; WGH: Wesley Guild Hospital.