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Published ahead of print on March 23, 2006, doi:10.1164/rccm.200601-125OC
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American Journal of Respiratory and Critical Care Medicine Vol 173. pp. 1264-1269, (2006)
© 2006 American Thoracic Society
doi: 10.1164/rccm.200601-125OC

Original Article

Abnormal Pulmonary Function in Adults with Sickle Cell Anemia

Elizabeth S. Klings, Diego F. Wyszynski, Vikki G. Nolan and Martin H. Steinberg

Pulmonary Center, Department of Medicine, Boston Comprehensive Sickle Cell Center, Boston University School of Medicine and School of Public Health, Boston, Massachusetts

Correspondence and requests for reprints should be addressed to Elizabeth S. Klings, M.D., Pulmonary Center, R-304, Boston University School of Medicine, 715 Albany Street, Boston, MA 02118. E-mail: eklings{at}lung.bumc.bu.edu

Rationale: Pulmonary complications of sickle cell anemia (Hb-SS) commonly cause morbidity, yet few large studies of pulmonary function tests (PFTs) in this population have been reported.

Objectives: PFTs (spirometry, lung volumes, and diffusion capacity for carbon monoxide [DLCO]) from 310 adults with Hb-SS were analyzed to determine the pattern of pulmonary dysfunction and their association with other systemic complications of sickle cell disease.

Methods: Raw PFT data were compared with predicted values. Each subject was subclassified into one of five groups: obstructive physiology, restrictive physiology, mixed obstructive/restrictive physiology, isolated low DLCO, or normal. The association between laboratory data of patients with decreased DLCO or restrictive physiology and those of normal subjects was assessed by multivariate linear regression.

Measurements and Main Results: Normal PFTs were present in only 31 of 310 (10%) patients. Overall, adults with Hb-SS were characterized by decreased total lung capacities (70.2 ± 14.7% predicted) and DLCO (64.5 ± 19.9%). The most common PFT patterns were restrictive physiology (74%) and isolated low DLCO (13%). Decreased DLCO was associated with thrombocytosis (p = 0.05), with hepatic dysfunction (elevated alanine aminotransferase; p = 0.07), and a trend toward renal dysfunction (elevated blood urea nitrogen and creatinine; p = 0.05 and 0.07, respectively).

Conclusions: Pulmonary function is abnormal in 90% of adult patients with Hb-SS. Common abnormalities include restrictive physiology and decreased DLCO. Decreased DLCO may indicate more severe sickle vasculopathy characterized by impaired hepatic and renal function.

Key Words: airway obstruction • dyspnea • restrictive disease




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