비부종양 자가면역망막병증 1예
A Case of Nonparaneoplastic Autoimmune Retinopathy
0
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
목적: 악성종양 동반 없이 발생한 자가면역망막병증을 혈청 항망막 자가항체 검사 등으로 진단한 1예를 보고하고자 한다. 증례요약: 60세 여자 환자가 3개월 이상의 진행성 양안 시력저하를 주소로 내원하였다. 최대교정시력은 우안 안전수동, 좌안 0.9였고, 전안부 및 안저검사상 양안의 뚜렷한 이상은 없었다. 자동시야검사상 양안의 심한 시야결손이 관찰되고, 망막전위도검사상 양안의암순응 b파의 심한 진폭저하를 보였으며, 빛간섭단층촬영상 황반부 광수용체의 내분절과 외분절 접합 부위가 불분명했다. 사람 망막단백질과 환자의 혈청으로 Western blot을 시행해 항망막 자가항체 존재를 확인하여 자가면역망막병증으로 진단하고, 후테논낭하스테로이드 주사, 전신 스테로이드 및 mycophenolate mofetil을 투여했다. 이후 망막전위도검사상 진폭이 소폭 상승했으나 주관적시력호전은 없었고, 빛간섭단층촬영검사에서도 변화가 없었다. 결론: 본 증례는 악성종양 동반 없이 발생한 자가면역망막병증을 항망막 자가항체 검사를 통해 진단한 국내 첫 보고이다. 안저이상은뚜렷하지 않으나 시야협착, 망막전위도 이상 등을 보이는 환자에서 자가면역망막병증을 감별해야 할 것으로 생각한다.
Abstract
Purpose: To report a case of nonparaneoplastic autoimmune retinopathy diagnosed using serum anti-retinal autoantibodies. Case summary: A 60-year-old female complained of progressive visual loss in both eyes over 3 months. Her best corrected visual acuity was hand motion in the right eye, 0.9 (decimal) in the left eye, and no definite abnormal findings were identified on fundus examinations. Automated visual field test revealed severely depressed visual fields in both eyes. Standard full-field electroretinogram (ERG) revealed nearly extinguished scotopic b-waves and spectral-domain optical coherence tomography (SD-OCT) showed subtle obscuration and interruption of the inner segment/outer segment (IS/OS) junction of the photoreceptors. Using Western blotting with human retinal proteins and the patient’s serum, we diagnosed nonparaneoplastic autoimmune retinopathy and performed posterior subtenon steroid injection in the right eye, systemic corticosteroids, and oral mycophenolate mofetil. Full-field ERG after treatment showed slightly increased amplitude but there was no subjective visual improvement. OCT after treatment did not reveal significant changes in the photoreceptor layer. Conclusions: This is the first reported case of nonparaneoplastic autoimmune retinopathy in Korea diagnosed using Western blotting with anti-retinal autoantibodies. Autoimmune retinopathy should be considered in patients with visual field and ERG impairment without definite fundus abnormalities.
참고문헌 (27)
- Comlekoglu DU., "Autoimmune retinopathy", Curr Opin Ophthalmol, 24, pp.598~605 (2013)
- Braithwaite T., "Autoimmune retinopathy", Ophthalmologica, 228, pp.131~142 (2012)
- Grange L., "Autoimmune retinopathy", Am J Ophthalmol, 157, pp.266~272.e1 (2014)
- Weleber RG., "Clinical and electrophysiologic characterization of paraneoplastic and autoimmune retinopathies associated with antienolase antibodies", Am J Ophthalmol, 139, pp.780~794 (2005)
- Grewal DS., "Autoimmune retinopathy and antiretinal antibodies : a review", Retina, 34, pp.1023~1041 (2014)
- Lima LH., "Hyperautofluorescent ring in autoimmune retinopathy", Retina, 32, pp.1385~1394 (2012)
- Abazari A., "Optical coherence tomography findings in autoimmune retinopathy", Am J Ophthalmol, 153, pp.750~756.e1 (2012)
- Faez S., "Concordance of antiretinal antibody testing results between laboratories in autoimmune retinopathy", JAMA Ophthalmol, 131, pp.113~115 (2013)
- Ko AC., "Anti-gamma-enolase autoimmune retinopathy manifesting in early childhood", Arch Ophthalmol, 128, pp.1590~1595 (2010)
- Pepple KL., "SD-OCT and autofluorescence characteristics of autoimmune retinopathy", Br J Ophthalmol, 97, pp.139~144 (2013)
- Mohamed Q., "Acute optical coherence tomographic findings in cancer-associated retinopathy", Arch Ophthalmol, 125, pp.1132~1133 (2007)
- Shimazaki K., "Frequency of anti-retinal antibodies in normal human serum", J Neuroophthalmol, 28, pp.5~11 (2008)
- Adamus G., "Antirecoverin antibodies and autoimmune retinopathy", Arch Ophthalmol, 118, pp.1577~1578 (2000)
- Adamus G., "Autoantibodies against retinal proteins in paraneoplastic and autoimmune retinopathy", BMC Ophthalmol, 4, pp.5~ (2004)
- Adamus G., "Autoantibody-induced apoptosis as a possible mechanism of autoimmune retinopathy", Autoimmun Rev, 2, pp.63~68 (2003)
- Mizener JB., "Autoimmune retinopathy in the absence of cancer", Am J Ophthalmol, 123, pp.607~618 (1997)
- Heckenlively JR., "Autoimmune retinopathy : a review and summary", Semin Immunopathol, 30, pp.127~134 (2008)
- Heckenlively JR., "Autoimmune retinopathy : patients with antirecoverin immunoreactivity and panretinal degeneration", Arch Ophthalmol, 118, pp.1525~1533 (2000)
- Heckenlively JR., "Investigations of antiretinal antibodies in pigmentary retinopathy and other retinal degenerations", Trans Am Ophthalmol Soc, 94, pp.179~200 (1996)
- Mrejen S., "Acute zonal occult outer retinopathy : a classification based on multimodal imaging", JAMA Ophthalmol, 132, pp.1089~1098 (2014)
- Or C., "Intravenous rituximab for the treatment of cancer-associated retinopathy", Can J Ophthalmol, 48, pp.e35~e38 (2013)
- Dy I., "Treatment of cancer-associated retinopathy with rituximab", J Natl Compr Canc Netw, 11, pp.1320~1324 (2013)
- Audemard A., "Melanoma-associated retinopathy treated with ipilimumab therapy", Dermatology, 227, pp.146~149 (2013)
- Rahimy E., "Paraneoplastic and non-paraneoplastic retinopathy and optic neuropathy : evaluation and management", Surv Ophthalmol, 58, pp.430~458 (2013)
- Chan JW., "Paraneoplastic retinopathies and optic neuropathies", Survey of ophthalmology, 48, pp.12~38 (2003)
- Ferreyra HA., "Management of autoimmune retinopathies with immunosuppression", Arch Ophthalmol, 127, pp.390~397 (2009)
- Heckenlively JR., "Controversies of diagnosing autoimmune retinopathy", Arch Ophthalmol, 128, pp.147~148 (2010)