Rinsho Ganka Volume 64, Issue 7 (July 2010)

Age-related macular degeneration in a case of hereditary angioneurotic edema Takayuki Nagai 1 , Yoriko Nakanishi 1 , Hitomi Kondo 1 , Tomoko Nagai 1 , Minoru Tanigawa 1 , Hiroyuki Yamamoto 1 1Dept of Ophthalmol,Nippon Steel Hirohata Hosp pp.1175-1181
Published Date 2010/7/15
DOI https://doi.org/10.11477/mf.1410103269
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Abstract. Background:Hereditary angioneurotic edema(HANE)is caused by complement activation of inherent deficiency of C1 inhibitor. Gene polymorphism of deficient C1 inhibitor has been reported in relation to age-related macular degeneration(AMD). Purpose:To report a case of HANE who developed unilateral AMD. Case:A 69-year-old male presented with impaired visual acuity in the right eye since 9 months before. He had been diagnosed with HANE with positive hematological findings before. Findings:Corrected visual acuity was 0.9 right and 1.0 left. The right eye showed subretinal fibrovascular proliferation in the paramacular area. He was diagnosed with AMD after visual acuity in the right eye deteriorated to 0.4 seven months later. Visual acuity improved to 1.0 fourteen months later following photodynamic therapy and intravitreal injection of ranibizumab. The left eye showed normal findings except paramacular drusen throughout. Conclusion:There is a possibility that complement activation by C1 inhibitor deficiency was associated with the onset of AMD in the present case.

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64巻7号 (2010年7月)
電子版ISSN 1882-1308 印刷版ISSN 0370-5579 医学書院