전신홍반루프스의 첫 발현 증상으로 발생한 양안의 심한 혈관폐쇄성 망막병증 환자 1예
A Case of Severe Vaso-Occlusive Retinopathy as the First Manifestation Associated with Systemic Lupus Erythematosus
2
조회
0
다운로드
1
인용
0
참고문헌
키워드
초록
목적: 양안 안전수동의 시력을 첫 주소로 내원하여 전신홍반루프스로 진단 받은 심한 혈관폐쇄성 망막병증 환자 1예를 보고하고자 한다. 증례요약: 23세 남자 환자로 양안의 시력저하를 주소로 진료의뢰 되었으며, 양안의 시력 안전수동으로, 교정되지 않았다. 형광안저혈관조영소견상 양안의 심한 망막출혈과 함께 면화반, 폐쇄성 혈관염, 망막부종 및 혈관구불거림 등의 소견이 관찰되었다. 전신검사상 양측뺨발진과 전신부종 소견을 보였고, 고혈압과 함께 질소혈증, 빈혈, 혈소판감소증을 보여 내과로 전과하여 항핵항체 강양성의 전신홍반루프스로 진단되었다. 이후 루프스 신염으로 전신 스테로이드 및 면역억제제치료, 혈액투석, 수혈치료 등을 받으며, 좌안에 유리체강내아바스틴 주입술 후 경과관찰하였다. 18개월 후 우안은 미만성 망막변성과 좌안은 견인력은 보이지 않는 망막전막만 남은채 양안시력이 우안은 안전수동, 좌안은 20/100으로 각각 유지되었다. 결론: 루프스 환자에서 혈관폐쇄성 망막병증은 비가역적인 심각한 시력저하를 가져올 수 있기 때문에, 루프스 망막병증의 위험요인이있는 환자에서 지속적인 안저검사와 전신질환에 대한 집중적인 관리가 필요할 것으로 생각한다.
Abstract
Purpose: To report a case of severe vaso-occlusive retinopathy with significant decrease of bilateral visual acuity as the first manifestation associated with systemic lupus erythematosus (SLE). Case summary: A 23-year-old man was referred to our clinic with bilateral visual impairment of hand motion (HH). Fundus examination revealed severe retinal hemorrhage, cotton-wool patch, occlusive retinal vasculitis with vascular engorgement,and diffuse retinal edema in both eyes. Because of a malar rash on both cheeks, generalized edema was observed on initial examination with hypertension, azotemia, anemia, and thrombocytopenia, The patient was diagnosed with SLE, strongly positive to antinuclear antibody (ANA), and received an intravitreal injection of Bevacizumab (Avastin, Genentech Inc.,San Francisco, CA, USA) in the left eye in addition to hemodialysis, transfusion, systemic corticosteroid and immunosuppressant treatment due to lupus nephritis. Eighteen months later, the retinal edema, cotton-wool patch and hemorrhage resolved,leaving epiretinal membrane without traction in his left eye and diffuse degeneration of the right eye. Final visual acuity was HM in the right eye and 20/100 in the left eye. Conclusions: Vaso-occlusive retinopathy in SLE can result in permanent visual impairment. In a patient with a high possibility of SLE retinopathy, a periodic fundus examination and intensive management of systemic disease should be considered.
참고문헌 (25)
- Gold DH, "Ocular findings in systemic lupus erythematosus", Br J Ophthalmol, 56, pp.800~804 (1972)
- Dougal MA, "Central retinal artery occlusion in systemic lupus erythematosus", Ann Ophthalmol, 15, pp.38~40 (1983)
- Hall S, "Occlusive retinal vascular disease in systemic lupus erythematosus", J Rheumatol, 11, pp.846~850 (1984)
- Graham EM, "Cerebral and retinal vascular changes in systemic lupus erythematosus", Ophthalmology, 92, pp.444~448 (1985)
- Jabs DA, "Severe retinal vaso-occlusive disease in systemic lupus erythematous", Arch Ophthalmol, 104, pp.558~563 (1986)
- Au A, "Review of severe vaso-occlusive retinopathy in systemic lupus erythematosus and the antiphospholipid syndrome: associations, visual outcomes, complications and treatment", Clin Experiment Ophthalmol, 32, pp.87~100 (2004)
- Giorgi D, "Retinopathy in systemic lupus erythematosus: pathogenesis and approach to therapy", Hum Immunol, 60, pp.688~696 (1999)
- Klinkhoff AV, "Retinopathy in systemic lupus erythematosus: relationship to disease activity", Arthritis Rheum, 29, pp.1152~1156 (1986)
- 윤창기, "전신홍반루프스 환자에서 발생한 망막병증", 대한안과학회지, 50(8), pp.1215~1220 (2009), 대한안과학회
- Quismorio FP Jr, "Clinical application of serologic abnormalities in systemic lupus erythematosus, In Dubois’ Lupus Erythematosus", pp.925~942 (1997), Lippincott Williams & Wilkins
- Mills JA, "Systemic lupus erythematosus", N Engl J Med, 330, pp.1871~1879 (1994)
- Stafford-Brady FJ, "Lupus retinopathy. Patterns, associations, and prognosis", Arthritis Rheum, 31, pp.1105~1110 (1988)
- Ingram SB, "An unusual syndrome of a devastating non-inflammatory vasculopathy associated with anticardiolipin antibodies: report of two cases", Arthritis Rheum, 30, pp.1167~1172 (1987)
- Montehermoso A, "Association of antiphospholipid antibodies with retinal vascular disease in systemic lupus erythematosus", Semin Arthritis Rheum, 28, pp.326~332 (1999)
- Asherson RA, "Antiphospholipid antibodies: a risk factor for occlusive ocular vascular disease in systemic lupus erythematosus and the ‘primary’ antiphospholipid syndrome", Ann Rheum Dis, 48, pp.358~361 (1989)
- Fitzpatrick EP, "The lupus anticoagulant and retinal vaso-occlusive disease", Ann Ophthalmol, 22, pp.148~152 (1990)
- Nag TC, "Vascular changes of the retina and choroid in systemic lupus erythematosus: pathology and pathogenesis", Curr Neurovasc Res, 3, pp.159~168 (2006)
- Gold D, "Retinal arterial occlusive disease in systemic lupus erythematosus", Arch Ophthalmol, 95, pp.1580~1585 (1977)
- Ushiyama O, "Retinal disease in patients with systemic lupus erythematosus", Ann Rheum Dis, 59, pp.705~708 (2000)
- Kleiner RC, "Vaso-occlusive retinopathy associated with antiphospholipid antibodies (lupus anticoagulant retinopathy)", Ophthalmology, 96, pp.896~904 (1989)
- Peponis V, "Ocular manifestations of systemic lupus erythematosus: a clinical review", Lupus, 15, pp.3~12 (2006)
- Kuryliszyn-Moskal A, "Vascular endothelial growth factor in systemic lupus erythematosus: relationship to disease activity, systemic organ manifestation, and nailfold capillaroscopic abnormalities", Arch Immunol Ther Exp, 55, pp.179~185 (2007)
- Kurup S, "Therapeutic efficacy of intravitreal bevacizumab on posterior uveitis complicated by neovascularization", Acta Ophthalmol, 87, pp.349~352 (2009)
- Lee WJ, "Intravitreal bevacizumab for severe vaso-occlusive retinopathy in systemic lupus erythematosus", Rheumatol Int (2011)
- Jung NH, "A case of severe retinal vaso-occlusive disease in systemic lupus erythematosus", J Korean Ophthalmol Soc, 34, pp.1287~1292 (1993)