Characteristics of patients with Fuchs endothelial corneal dystrophy in Japanese national registry

Abstract

Purpose : To analyze the characteristics of patients with Fuchs corneal endothelial dystrophy (FECD) registered in Japanese national registry established by the Ministry of Health, Labour and Welfare intractable disease policy group and AMED practical application research project group for intractable diseases.

Methods : We analyzed clinical information in the registry on Rare Disease Data Registry of Japan by means of medical records and interviews. The mean, standard deviation, and ratio of each item were calculated excluding cases registered as unknown.

Results : Information on 512 patients was registered from 11 facilities. The average age was 67.3 ± 13.0 years for male and 70.2 ± 11.3 years for female. The average body mass index was 23.8 ± 6.1 for male and 22.6 ± 3.4 for female. There was a history of outdoor work in 2.9%, a history of contact lens wear in 8.9%, a history of smoking in 14.1%, diabetes in 12.8%, hypertension in 33.8%, and glaucoma in 15.4%. Phakic eyes were 63.4%, and pseudophakic eyes were 36.6%. As subjective symptoms, 64.1% of the patients were aware of visual disturbance, 39.8% had photophobia, 18.9% had morning blur compared to night, and 4.7% had ocular pain. The average corrected distance visual acuity was 0.30 ± 0.47 logMAR. The average central corneal thickness was 601.8 ± 83.9 um. And, 216 eyes (21.1%) had a history of keratoplasty.

Conclusions : The national registry of FECD patients allowed us to understand the characteristics of patients with FECD followed by cornea specialists in Japan.

This abstract was presented at the 2024 ARVO Annual Meeting, held in Seattle, WA, May 5-9, 2024.