Abstract
BackgroundSickle cell disease (SCD) is a multisystem disorder characterized by a wide spectrum of clinical manifestations and severity. Studies investigating potential effects of co-morbid human immunodeficiency virus (HIV) and SCD have produced conflicting results, and additional investigations are needed to elucidate whether the interaction between the two disease states might impact both HIV and SCD clinical outcomes. The association of HIV infection with clinical and laboratory characteristics of patients with SCD was assessed.MethodsThis nested case-control study included individuals with SCD with HIV treated at six Brazilian SCD centers. Clinical and laboratory data were abstracted from medical records. HIV positive participants were compared to age, gender, center, and SCD genotype matched HIV negative participants (ratio 1:4). Individual clinical outcomes as well as a composite outcome of any SCD complication and a composite outcome of any HIV-related complication were compared between the two groups.ResultsFifteen HIV positive participants were included, 12 (80%) alive and 3 (20%) deceased. Most of the HIV positive patients had HbSS (60%; n = 9), 53% (n = 8) were female, and mean age was 30 ± 13 years. The frequency of individual SCD complications of acute chest syndrome/pneumonia, sepsis/bacteremia, pyelonephritis, ischemic stroke, hemorrhagic stroke, abnormal transcranial Doppler (TCD), and pulmonary hypertension was higher in HIV positive participants when compared to HIV negative, although analyzed individually none were statistically significant. HIV positive participants had significantly higher risk of any SCD complication and of a composite HIV-related complication compared to the HIV negative group (HR = 4.6; 95%CI 1.1–19.6; P = 0.04 and HR = 7.7; 95%CI 1.5–40.2; P = 0.02, respectively). There was a non-significant trend towards higher risk of any infections in participants with HIV positive (HR = 3.5; 95%CI 0.92–13.4; P = 0.07). Laboratory parameters levels were not significantly different in individuals with and without HIV.ConclusionsIn summary, our study in SCD patients shows that those with HIV have an increased risk of any SCD complication and HIV-related complications, as well as a suggestive but not significantly increased risk of infections.
Highlights
Sickle cell disease (SCD) is a multisystem disorder characterized by a wide spectrum of clinical manifestations and severity
The frequency of individual SCD complications of acute chest syndrome/pneumonia, sepsis/bacteremia, pyelonephritis, ischemic stroke, hemorrhagic stroke, abnormal transcranial Doppler (TCD), and pulmonary hypertension was higher in human immunodeficiency virus (HIV) positive participants when compared to HIV negative, analyzed individually none were statistically significant
HIV positive participants had significantly higher risk of any SCD complication and of a composite HIVrelated complication compared to the HIV negative group (HR = 4.6; 95%CI 1.1–19.6; P = 0.04 and HR = 7.7; 95%CI 1.5–40.2; P = 0.02, respectively)
Summary
Sickle cell disease (SCD) is a multisystem disorder characterized by a wide spectrum of clinical manifestations and severity. In the last three decades, advances in diagnosis and treatment have led to considerable progress in the life expectancy of patients with HIV [6], as well as in those with SCD [1]. Both diseases have become chronic illnesses, especially in developed countries. One systematic review suggested that HIV may worsen symptoms of SCD [7] Both HIV and SCD are independent risk factors for certain outcomes such as stroke, avascular necrosis, pulmonary hypertension, kidney disease and infections. It is possible that HIV and SCD may interact to increase the risk of these complications
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