Clinical and laboratory factors associated with the severity of proliferative sickle cell retinopathy in patients with sickle cell hemoglobin C (SC) and homozygous sickle cell (SS) disease.

Proliferative sickle cell retinopathy (PSCR) is the most frequent vision-threatening complication of sickle cell disease (SCD). We investigated the relationship between the severity of sickle cell retinopathy in heterozygous (SC) or homozygous (SS) adult SCD patients and the clinical and laboratory data obtained during visits to a national SCD referral center. This retrospective longitudinal analysis included 942 SCD patients (313 patients with SC and 629 with SS disease) with ophthalmologic evaluations who were followed over a 19-year period by a multidisciplinary team in a referral center. PSCR was graded using the Goldberg classification. We identified patient and SCD characteristics associated with sickle cell retinopathy severity using multinomial logistic-regression models. Multivariate analysis associated severe PSCR forms (stages III-V) with older age (p=0.032), pulmonary involvement (documented pulmonary hypertension with pulmonary arterial pressure≥40 mm Hg, restrictive syndrome>20%, or previous history of pulmonary embolism diagnosed by vascular imaging) (p=0.029), deafness or tinnitus (p=0.026), and no history of osteomyelitis (p=0.013) for SC patients; and with older age (p<0.001), male sex (p=0.003), and acute pyelonephritis (p=0.04) for SS patients. The model of severe PSCR versus no PSCR showed good calibration and discrimination for SC and SS patients. Awareness of the clinical and laboratory factors significantly associated with severe PSCR in patients with SC or SS SCD may contribute to improved preventive strategies.

Risk factors for the development of ocular complications of Stevens-Johnson syndrome and toxic epidermal necrolysis.

OBJECTIVES: To describe the acute and late ocular manifestations of toxic epidermal necrosis (TEN), Stevens-Johnson syndrome (SJS), and overlap syndrome and to identify predictors for the development of ocular complications. DESIGN: Retrospective cohort study. SETTING: A single referral unit in a university hospital. PATIENTS: The study included 159 patients (mean [SD] age, 49.9 [19.8] years) with TEN and SJS during an 8-year period. Forty-nine patients were contacted at least 15 months after hospital discharge. MAIN OUTCOME MEASURES: Records were reviewed for demographics, cause of the condition, and severity of ocular involvement. The patients were contacted to assess late ocular complications. RESULTS: A total of 117 patients (74%) had acute ocular involvement, which was mild in 58%, moderate in 8%, and severe in 8%. Patients with TEN had more frequent (odds ratio [OR], 2.7; 95% confidence interval [CI], 1.06-6.90; P = .05) but not more severe (OR, 0.95; 95% CI, 0.20-4.5; P = .99) acute ocular involvement. Forty-nine patients were contacted at least 15 months after hospital discharge, and 63% had late ocular complications. Dry eye syndrome was the most common. The mean (SD) Ocular Surface Disease Index score was 32.9 (30.3) (range, 0-97.5). The severity of the acute ocular disease was found to be the only significant risk factor of late complications (P = .002), even though 5 patients without acute ocular involvement developed dry eye syndrome. CONCLUSIONS: Ocular involvement is common in patients with SJS and TEN. Late complications are more frequent in patients with severe initial eye involvement but may also develop in patients without patent initial ocular symptoms.