Lung Function Abnormalities in Sickle Cell Anaemia

Area of Science:

• Pulmonary Medicine
• Hematology

Background:

• Lung function abnormalities are common in sickle cell disease (SCD) patients, even during steady state.
• Prevalence and patterns of these abnormalities are not well-characterized in all SCD cohorts.
• Limited information exists on risk factors and clinical relevance of lung function deficits in SCD.

Purpose of the Study:

• To investigate the prevalence and patterns of lung function abnormalities in adult Hb-SS patients.
• To compare lung function between hydroxyurea-naive adult Hb-SS patients and non-SCD controls.
• To explore associations between lung function abnormalities and clinical markers in SCD.

Main Methods:

• Analytical cross-sectional study of 76 clinically stable, hydroxyurea-naive adult Hb-SS participants and 76 non-SCD controls.
• Data collection included sociodemographic information, clinical history, spirometry, pulse oximetry, and echocardiography (TRV).
• Laboratory tests included complete blood counts, free plasma hemoglobin, serum urea, and creatinine.

Main Results:

• Hb-SS patients had significantly lower weight, BMI, FVC, and FEV1% predicted values compared to controls (p < 0.001).
• 70.4% of Hb-SS patients showed abnormal spirometry, predominantly restrictive defects (p < 0.001).
• Lower mean oxygen saturation was observed in Hb-SS patients (p < 0.001); no significant association was found with Hb, WBC, free plasma hemoglobin, crisis frequency, leg ulcers, or TRV.

Conclusions:

• Lung volumes are significantly reduced in Hb-SS patients versus non-SCD controls, independent of anthropometric factors.
• Restrictive lung defects are highly prevalent in Hb-SS patients.
• These lung function abnormalities in SCD are not significantly associated with established markers of disease severity.