Progression Rate of Visual Function and Affecting Factors at Different Stages of Retinitis Pigmentosa

Abstract

We reviewed medical records of 121 patients/235 eyes of typical retinitis pigmentosa (RP) patients who could be followed up for at least 5 years with the aim of investigating the long-term course of visual function progression at each RP stage and appropriate assessment methods. Patients were classified into three groups: mild RP ( $\text{baseline}\text{mean}\text{deviation}\left(\text{MD}\right)\ge -5$ ), moderate RP ( $-25<\text{baseline}\text{MD}<-5$ ), and late RP ( $\text{baseline}\text{MD}\le -25$ ). Linear mixed-effect models were used to follow MD, the average retinal sensitivity of the central four points of the Humphrey field analyzer 10-2 program (S4), and visual acuity (VA) with increasing time. The associations among factors (baseline MD group, sex, hereditary form) and the interaction between each factor and time were also investigated. The mean reduction of the MD, S4, and VA for all patients was -0.37 dB/year, -0.25 dB/year, and 0.018/year, respectively. The moderate RP group had a faster progression than other groups in MD (-0.43 dB/year, $p<0.05$ ). The moderate (-0.31 dB/year, $p=0.01$ ) and late RP groups (-0.25 dB/year, $p<0.01$ ) had faster progression than the mild RP group in S4. The late RP group had faster progression in VA than the other groups (0.03/year, $p<0.05$ ). Females had a slower progression of the S4 (-0.15 dB/year, $p=0.02$ ) and VA (0.01/year, $p<0.001$ ) than males. The autosomal dominant group had a slower progression than the sporadic group in MD (-0.22 dB/year, $p=0.02$ ); the autosomal dominant and autosomal recessive groups had a slower VA decline than the sporadic group (0.01/year, $p=0.03$ ; 0.01/year, $p=0.04$ ). Because the progression rates of VA and visual field test differed as per the RP stage, S4 and VA can also be useful assessment methods depending on the stage. Inheritance form and sex may affect the progression rate.