Risk Factors of Lorch et al for Excess Mortality in an Adult with Sickle Cell Disease

 

Independent predictors of mortality identified on multivariate analysis:

(1) estimated pulmonary arterial systolic pressure (ePASP) >= 40 mm Hg at any time (hazard ratio 7)

(2) estimated glomerular filtration rate (GFR) < 60 mL per min per 1.73 square meters BSA (hazard ratio 16)

(3) total serum bilirubin > 5 mg/dL (hazard ratio 9)

 

where:

• Pulmonary hypertension in SCD is discussed in 08.14.13.

• Estimating the pulmonary arterial systolic pressure from the tricuspid regurgitant jet velocity is discussed in 07.20.14.

• The elevated total serum bilirubin may reflect hemolysis and/or sickle cell hepatopathy.

 

A higher level of hemoglobin F may be marginally protective (hazard ratio 0.8).

 

The elevated pulmonary arterial systolic pressure and decreased GFR reflect the vasculopathy associated with sickle cell disease.