Adults with sickle cell disease who actively smoke cigarettes or cigars are nearly three times as likely to experience advancing sickle cell retinopathy compared to nonsmokers.
RT’s Three Key Takeaways:
- Heightened Progression Risk: Adults with sickle cell disease who actively smoke cigarettes or cigars are nearly three times as likely to experience advancing sickle cell retinopathy compared to nonsmokers.
- Actionable Intervention Point: Because current smoking was linked to disease worsening while past smoking history was not, smoking cessation presents an immediate, modifiable clinical strategy to help preserve patient vision.
- Importance of Regular Screening: Routine eye examinations starting in late childhood remain critical for detecting retinal vessel changes before irreversible visual impairment develops.
Active smoking is a major modifiable risk factor for the progression of sickle cell retinopathy (SCR), a vision-threatening condition affecting individuals with sickle cell disease (SCD), according to a 10-year retrospective study published in the American Journal of Ophthalmology.
Investigators from the Wilmer Eye Institute at Johns Hopkins Medicine evaluated long-term outcomes to clarify the relationship between tobacco use and ocular vascular damage. The study is believed to be the largest US-based longitudinal investigation of adults with SCR that used modern medical testing and imaging to track disease progression over time.
“Sickle cell retinopathy is severely understudied, and data is needed to create hypotheses to fund research that helps clinicians understand it,” said Adrienne Scott, MD, study principal investigator and professor of ophthalmology at the Johns Hopkins University School of Medicine. “When we started our work, we realized even basic information, like SCR risk factors, couldn’t be definitively stated. Because of this, we decided to determine them ourselves.”
Longitudinal Findings on Retinopathy Progression
SCR occurs when rigid, misshapen red blood cells obstruct retinal microvasculature. In the study, researchers reviewed records for 317 adult patients, representing 620 eyes, who had an established SCD diagnosis and were evaluated between July 1, 2013, and June 30, 2023. The cohort had a mean age of 35 years and included 336 eyes with non-proliferative SCR and 284 eyes with proliferative SCR, which is characterized by abnormal, fragile neovascularization that can cause severe vision loss.
According to the study data, 36 eyes (10.7%) initially diagnosed with non-proliferative SCR progressed to proliferative disease over eight years of follow-up. Among eyes presenting with proliferative SCR at baseline, further disease progression occurred in 44 eyes (17.0%) over 11 years.
When analyzing underlying risk variables, the researchers found that patients who actively smoked cigarettes or cigars at the time of proliferative SCR diagnosis had nearly three times the hazard of disease progression compared to nonsmoking counterparts (hazard ratio, 2.78). Conversely, the authors reported no statistically significant links between disease progression and age at diagnosis, sex, SCD genotype, hydroxyurea therapy, blood transfusions, chronic red cell exchanges, bone marrow transplantation, or lifetime smoking history.
“Smoking has been implicated in many different eye diseases, such as age-related macular degeneration, and we believe this is the first study showing a clear link between active smoking and sickle cell retinopathy progression. With this knowledge, clinicians and patients now have a direct and actionable means of preserving the patient’s eye health,” said Anshuman Agrawal, study lead author and medical student at the Johns Hopkins University School of Medicine.
Clinical Implications for Healthcare Providers
The study authors noted that active tobacco consumption appears to be a stronger driver of retinal progression than prior tobacco exposure, suggesting that cessation initiatives can provide meaningful benefits regardless of past smoking habits.
For multidisciplinary healthcare teams, including respiratory therapists and chronic disease specialists who regularly engage SCD patients in smoking cessation counseling, these findings highlight tobacco avoidance as an actionable strategy to reduce systemic microvascular morbidity.
The investigators emphasized that annual retinal examinations beginning at age 10 remain essential, as SCR frequently develops without early symptoms. Although the study population from a tertiary referral institution may reflect more severe disease than the broader SCD population, the authors indicated that earlier identification and targeted risk reduction can help reassure patients.
“I want to reassure people with sickle cell disease that, even with sickle cell retinopathy, they have a high chance of having normal vision for a long time. But treating sickle cell disease and attending regular retina exams is key to preserving sight, if it does develop,” said Scott.
