Impact of liver transplantation on transthyretin-related ocular amyloidosis in Japanese patients.
OBJECTIVE
To evaluate the long-term impact of liver transplantation on ocular manifestations of familial amyloid polyneuropathy (FAP) in Japanese patients.


METHODS
Medical records were retrospectively reviewed in a long-term follow-up study. Of 52 patients with FAP amyloidogenic transthyretin Val30Met, 22 patients underwent liver transplantation. We assessed ocular manifestations, including amyloid deposition at the pupillary border, pupillary border with irregularity, vitreous opacities, and glaucoma, in patients who underwent liver transplantation. In addition, we compared the clinical characteristics of vitreous opacities-the most common ocular manifestation of FAP-in patients who underwent liver transplantation and those who did not to determine the effect of transplantation on the progression of ocular amyloidosis.


RESULTS
Mean time after FAP onset was 10 years and after liver transplantation was 7 years in patients who underwent liver transplantation. All ocular manifestations increased with time after transplantation. Eight patients (36%) developed vitreous opacities and 4 patients (18%) developed glaucoma during follow-up. Mean time from FAP onset to vitreous opacities onset was significantly shorter in patients with early-onset disease who underwent liver transplantation than in those who did not.


CONCLUSIONS
Patients with FAP who undergo liver transplantation continue to have a long-term risk of severe ocular manifestations, especially vitreous opacities and glaucoma, which can restrict their daily lives, even after liver transplantation.
Department of Ophthalmology and Visual Science, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto 860-8556, Japan.
Ryuhei
R
Hara
Takahiro
T
Kawaji
Eiko
E
Ando
Yuki
Y
Ohya
Yukio
Y
Ando
Hidenobu
H
Tanihara
D000328:Adult; D017668:Age of Onset; D000368:Aged; D000682:Amyloid / Q000378:metabolism*; D028227:Amyloid Neuropathies, Familial / Q000208:ethnology / Q000503:physiopathology / Q000601:surgery*; D000686:Amyloidosis / Q000208:ethnology / Q000378:metabolism / Q000503:physiopathology*; D044466:Asian People / Q000208:ethnology; D005128:Eye Diseases / Q000208:ethnology / Q000378:metabolism / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007499:Iris Diseases / Q000208:ethnology / Q000378:metabolism / Q000503:physiopathology*; D007564:Japan / Q000453:epidemiology; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D011228:Prealbumin / Q000378:metabolism*; D012189:Retrospective Studies; D014822:Vitreous Body / Q000378:metabolism / Q000473:pathology*
D000682:Amyloid; D011228:Prealbumin; C076571:transthyretin-related amyloid fibril protein, human