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1 대한안과학회지 2017 년제 58 권제 10 호 J Korean Ophthalmol Soc 2017;58(10): ISSN (Print) ISSN (Online) Original Article 육아종증다발혈관염의눈및눈부속기관침범에대한후향적분석 A Retrospective Analysis of Granulomatosis with Polyangiitis with Ocular Manifestations 김보람 서유리 윤진숙 Bo-ram Kim, MD, Yuri Seo, MD, Jin Sook Yoon, MD, PhD 연세대학교의과대학안과학교실시기능연구소 The Institute of Vision Research, Department of Ophthalmology, Yonsei University College of Medicine, Seoul, Korea Purpose: To analyze the treatment and prognosis of patients from our tertiary medical center with ocular and orbital involvement of granulomatosis with polyangiitis. Methods: A retrospective analysis of the medical records of patients diagnosed with granulomatosis with polyangiitis that visited our single tertiary referral center from July 2008 to September 2014 was performed. Results: A total of 51 patients diagnosed with granulomatosis with polyangiitis visited our center, and 21 of those patients had received an ophthalmologic examination. Of these, 9 patients (4 males, 5 females) had symptoms of the eye and orbit, and the clinical presentations were as follows: episcleritis, scleritis, marginal keratitis, orbital inflammation, orbital abscess, retinal vasculitis, and nasolacrimal duct obstruction. The patients each received treatments according to clinical presentation with topical, oral, or intravenous steroids or immunomodulatory agents such as cyclophosphamide. Nasolacrimal duct obstruction was treated with surgery in some cases. After an average follow-up period of 58 ± 30 months, all patients showed clinical improvement of their ocular and orbital involvement of granulomatosis with polyangiitis. Conclusions: Granulomatosis with polyangiitis is a relatively rare disease that sometimes has ocular or orbital involvement and can lead to blindness. Therefore, when ocular symptoms and signs present without a definitive cause, granulomatosis with polyangiitis must be ruled out, and appropriate treatment is needed. However, there are few published reports on the clinical presentation and prognosis of ocular and orbital involvement of granulomatosis with polyangiitis in Asians. This study showed that the incidence of ocular and orbital involvement in granulomatosis with polyangiitis was lower than previous reports. J Korean Ophthalmol Soc 2017;58(10): Keywords: Granulomatosis with polyangiitis, Ocular involvement, Wegener s granulomatosis 육아종증다발혈관염 (granulomatosis with polyangiitis) 은이전에베게너육아종증 (Wegener s granulomatosis) 으 Received: Revised: Accepted: Address reprint requests to Jin Sook Yoon, MD, PhD Department of Ophthalmology, Severance Hospital, #50-1 Yonsei-ro, Seodaemun-gu, Seoul 03722, Korea Tel: , Fax: yoonjs@yuhs.ac * Conflicts of Interest: The authors have no conflicts to disclose. 로불리었던질환으로, 1 주로작거나중간크기의혈관을침범하는괴사성혈관염및호흡기, 신장을주로침범하는육아종성염증을특징으로하는자가면역질환이다. 2 미국류마티스학회에서제안한육아종증다발혈관염의분류기준에따르면 (1) 비강또는구강의염증, (2) 결절, 공동, 고정된침윤을보이는비정상적인흉부방사선영상, (3) 5 개이상의적혈구또는적혈구원주를포함하는비정상적인요침전물, (4) 동맥혹은혈관주위의조직검사상육아종성염증소견이포함되어있으며, 이중 2가지이상에해당할경우진단할수있다. 3,4 또한, 진단기준에는포함되어 c2017 The Korean Ophthalmological Society This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 1115

2 - 대한안과학회지 2017 년제 58 권제 10 호 - 있지않지만, 활동성의육아종증다발혈관염환자들의 90% 가까이에서세포질항중성구세포질항체 (cytoplasmic antineutrophil cytoplasmic antibody, c-anca); 단백분해효소 3 (proteinase3, PR3) 양성으로나타나며, 3% 에서는핵주위항중성구세포질항체 (perinuclear antineutrophil cytoplasmic antibody, p-anca); 골수세포형과산화효소 (myeloperoxidase, MPO) 양성으로나타난다. 5,6 육아종증다발혈관염은약 50-60% 에서눈및눈부속기관침범이일어나는것으로알려져있으며, 연구에따라 87% 까지보고되기도한다. 7,8 결막염, 상공막염등경한질환부터각막염, 망막혈관염, 망막혈관폐쇄, 염증성안와질환등시력저하를일으키는중한질환, 그리고비루관폐쇄등수술이필요한질환등다양한양상으로나타나는데, 8% 에서는시력을잃을수도있다. 9 그러나진단당시눈및눈부속기관의이상증상을보이거나또는눈및눈부속기관의이상증상이질병의첫발현증상인경우는 8-16% 로보고되었다. 7 현재까지다발성육아종증의증례나보고논문은대부분서양인대상으로발표되고있으며, 이전연구에서국내의 3차단일의료기관의환자를대상으로육아종증다발혈관염의안과적발현과치료, 그리고그결과에대한보고는이루어진바없어본연구를통해이를확인하고자한다. 대상과방법 2008년 7월부터 2014년 6월까지연구에참여한단일의료기관에내원한환자중미국류마티스학회에서제안한육아종증다발혈관염의분류기준에부합하여 3,4 이를진단받은환자에대하여후향적의무기록조사를진행하였으며, 그중눈증상을호소하여안과외래에내원하였던환자들의안과진료기록을분석하였다. 육아종증다발혈관염환자들은모두임상양상에따라내과적및안과적치료를시행받았다. 본연구는본원의연구윤리심의위원회 (Institutional Review Board, IRB) 의승인을받았다 ( ). 육아종증다발혈관염의진단은미국류마티스학회의기준에따랐으며, 육아종증다발혈관염의눈증상 ( 결막염, 상공막염, 각막염, 망막혈관염, 비루관폐쇄등 ) 을나타내는환자중의무기록열람이가능하고, 치료를받은뒤 12 개월이상추적관찰이가능한환자들을대상으로자료를분석하였다. 나이, 성별, 과거력, 시력, 안압, 안과및타과진단명, 진단에대한치료 ( 안약, 스테로이드사용유무, 방사선치료, 수술적치료등 ), 육아종증다발혈관염의유병 기간, p-anca, c-anca 등혈액검사결과, 기타장기침범여부등을조사하고기존해외연구결과와비교분석하였다. 결과 2008년 7월부터 2014년 6월까지본원을방문한육아종증다발혈관염환자는총 51명이고이들중눈및눈부속기관의이상증상을호소하여안과외래에내원한환자는 21명이었다. 이들중 9명 (18%) 이눈및눈부속기관침범이있었다. 눈및눈부속기관침범이있는육아종증다발혈관염환자의성비는남자 4명, 여성 5명이었고, 평균 57 ± 14세였으며, 경과관찰기간은평균 58 ± 30개월이었다. 이중 6명의환자들은육아종증다발혈관염외다른내과적질환을동반하였다 ( 고혈압 3명, 갑상선항진증 1명, 고감마글로불린혈증 1명, 종양 2명 ). 면역학적검사로는 8명 (89%) 의환자군에서 c-anca 양성을보였으며, 1명 (11%) 의환자에서는음성을보였다. 또한 2명 (22%) 의환자군에서 p-anca 양성을보였으며, 7명 (77%) 의환자에서는음성을보였다. 총 7명의환자들 ( 폐침범 6명, 신장침범 4명, 뇌침범 2 명 ) 에게서전신적인염증소견이있었고, 이들은모두전신적인경구혹은고용량경정맥스테로이드치료요법을받았으며, 이중 6명은사이클로포스파미드 (cyclophosphamide) 등의전신적인면역억제제를추가로복용하였다. 본연구에서안구돌출과복시증상을나타낸증례와비루관침범으로인한눈물흘림과안와연조직염의증소견으로나타난증례, 총 2개 (4%) 의증례에서눈및눈부속기관의이상증상을통해육아종증다발혈관염을진단하였다. 이 2개의증례에서모두영상검사에서안와및비강내의이상병변이발견되었고, 생검을통해육아종증다발혈관염을진단하였다. 눈및눈부속기관침범증상으로는상공막염 (2%), 공막염 (2%), 망막혈관염 (2%), 주변부각막염 (2%), 염증성안와질환 (6%), 안와농양 (2%), 비루관폐쇄 (6%) 등이있었고, 안과내원당시육아종증다발혈관염의눈및부속기관침범을통해질병의전신적증상관해후재발된경우가 3명 (6%) 이었으며, 이를통해질병이처음진단된경우는 2명 (4%) 인것으로나타났다. 국소스테로이드제점안, 경구스테로이드, 면역억제제, 경정맥스테로이드주사및수술등의치료를시행하였고, 모든환자에서치료후눈및눈부속기관의이상증상의임상적호전을보였다 (Table 1-3, Fig. 1). 1116

3 - 김보람외 : 육아종증다발혈관염의후향적분석 - 고 찰 육아종증다발혈관염은국소적인육아종으로시작하여혈관염의형태로발현되는전신성질환으로추정된다. 2 이는상 하기도및폐를침범하는임상양상이가장흔하며, 여러장기를침범하기도한다. 현재까지발표된대부분의논문들은서양인을대상으로한연구들로, 약 50-60% 정도의눈및부속기관의침범률을보이며, 7,8 본연구에서는이에못미치는약 18% 의침범률을보이고있다. 육아종증다발혈관염의치료로는고용량경정맥스테로이드요법, 사이클로포스파미드등의면역억제제가사용되고있으며, 최근에는리툭시맙 (rituximab) 및항종양괴사인자알파 (anti tumor necrosis factor alpha, anti TNF-α) 를이용한치료에대해연구가활발히이루어지고있다. 10,11 유럽류마티스학회 (European League Against Rheumatism, EULAR) 에서관해는 7.5 mg 이하의 prednisolone 또는면역억제제복용없이도질병이비활동성을유지하는경우로, 재발은질병이다시활동성이되거나새로운증상이나타나는경우로정의하고있다. 12 따라서추적관찰기간중질병의치료효과를판단하기위해서는질병활성도를확인하는것이중요한데, 안과적으로는전안부의경우세극등검사를통한임상소견과질환에따라정립된 grading scale을이용할수있겠고, 구후부나비강의경우에는영상학적검사등으로그변화를판단해야하겠으며, 혈액검사를통해 ANCA의음전여부를확인하는것또한도움이될수있겠다. ANCA의경우질병의활성도와는연관이있으나, 관해와는상관관계가없기때문에관해가이루어진후육아종증다발혈관염이다시재발하는것에관련된지표를알고자하는여러연구들이현재진행되고있다. 13 진단당시눈및눈부속기관의이상증상을보이거나또는눈및눈부속기관의이상증상이질병의첫발현인경우는 8-16% 정도로드물게보고되고있으며 7 본연구에서는관해상태후눈및부속기관침범을통해재발이이루어진경우가 3명 (6%), 눈및눈부속기관의이상증상이질병의첫발현인경우역시 2명 (4%) 으로나타났다. 국내의경우망막색소상피박리와망막하출혈을동반한육아종증다발혈관염환자 1예 14 와안과적합병증을동반한육아종증다발혈관염환자 5예 15 등이보고된바있으나대부분인후두증상이나다른전신질환을동반하고있었으며안구침범이초기증상으로나타난경우는아니었다. 현재까지가장장기간으로연구된육아종증다발혈관염의코호트연구는총 158명의환자군을대상으로이루어졌으며, 이는미국에서행해진연구로 97% 의백인으로구성되어있다. 이에따르면전체육아종증다발혈관염환 1117

4 - 대한안과학회지 2017 년제 58 권제 10 호 - Table 2. Ocular and systemic treatment of patients with granulomatosis with polyangiitis Patient No. Ophthalmic manifestation Treatment for eye Systemic treatment 1 Episcleritis (OU) Topical steroid Steroid pulse (62.5 mg/day 3 wks mg/day) Pre VA (OD/OS) UC/UC Post VA (OD/OS) UC/UC 2 Scleritis (OU) Topical antibiotics PO steroid (5-10 mg/day) PO steroid (5-10 mg/day) Immunosuppressive agent (Azathioprine) 0.9/ /0.5 3 Retinal vasculitis (OD) Peripheral keratitis (OD) Retinal photocoagulation (OD) PO steroid (5-15 mg/day) PO steroid (5-15 mg/day) Immunosuppressive agent (Cyclophosphamide Azathioprine), Lung resection, Transsphenoidal surgery 0.9/ /0.9 4 Orbital inflammation (OS) IV steroid (3.75 g) PO steroid (25-40 mg) IV steroid (3.75 g) PO steroid (25-40 mg) Immunosuppressive agent (Cyclophosphamide) 0.8/ /0.4 5 Dacryoadenitis (OU) Topical antibiotics and steroid PO steroid (5-30 mg/day) Steroid pulse (1 g) PO steroid (5-30 mg/day) Immunosuppressive agent (Cyclophosphamide) d/c 0.8/0.8-6 Orbital inflammation (OU) NLD obstruction (OU) IV steroid (Medrol 12.5 g, Cortef 400 mg) Endoscopic DCR (OU) The operation was done during remission Steroid pulse (4 times) PO steroid (5-60 mg/day) Immunosuppressive agent (Cyclophosphamide, Mycophenolate mofetil) 0.9/ /1.0 7 NLD obstruction (OU) Topical antibiotics and steroid Immunosuppressive agent (Cyclophosphamide pulse 10 times Azathioprine mg/day) PO steroid 2-10 mg/day 0.8/ /0.7 8 NLD obstruction (OU) Orbital cellulitis IV steroid (1 g) Endoscopic DCR (OU) The 1st operation was done 2 weeks after IV steroid pulse therapy and the revision operation was done during remission IV antibiotics PO steroid ( mg/day) Immunosuppressive agent (Methotrexate) 0.6/ /0.8 9 Orbital abscess (OD) Subperiosteal abscess drainage (OD) None (antibiotic therapy was initiated due to sign of the infection, but the follow up loss occurred.) 0.5/0.5 (near) 0.7/0.7 VA = visual acuity; OD = oculus dexter; OS = oculus sinister; OU = oculus unitas; wks = weeks; UC = uncheckable; PO = per oral; IV = intravenous; d/c = discontinue; NLD = nasolacrimal duct; DCR = dacryocystorhinostomy. 자중약 52% 환자에서안와침범이일어났으며, 약 8% 환자에서영구적인시력저하로이어졌다는결과를볼수있다. 또한공막염 (7%), 상공막염 (3.5%), 각막침범 (8%), 비루관 (7%) 등다양한임상양상으로나타나는것을알수있다. 9 본연구는총 6년간의 3차단일의료기관에서한국인을대상으로이루어진후향적연구로써, 이전에발표된해외연구결과들에못미치는약 18% 에해당하는환자들에게서눈및부속기관의침범이발생한것을확인할수있었으며, 상공막염 (2%), 공막염 (2%), 염증성안와질환 (6%), 안와농양 (2%), 망막혈관염 (2%), 비루관폐쇄 (6%) 등다양한임상양상으로나타나는것을볼수있었다. 일반적으로안구돌출은육아종증다발혈관염의눈및눈부속기관침범이일어난환자의약 15% 에발생하는데, 외안근에침범할경우복시증상을나타내며, 심한경우압박성시신경경색을일으켜실명까지이르게된다. 9 본연구에서는 4명 (8%) 의환자군에서안구돌출및복시의증상을나타냈으며 ( 안구돌출 3명, 복시 2명 ), 그중 1명 (2%) 에게서시행한안와전산화단층촬영상안와연부조직의조영증가양상을확인할수있었다. 비루관쪽의침범이일어나는경우는약 7-10% 로보고되고있으며, 10 본연구에서는 3명 (6%) 의환자에게서눈물흘림증상을통해비루관침범을발견하였다. 또한육아종증다발혈관염이공막염혹은상공막염의형태로눈및눈부속기관침범이나타날수있는데, 약 10% 의환자에게서나타나는것으로알려져있다. 9,16 본연구에서는총 2명 (4%) 의환자에게서공막염및상공막염의형태로눈및눈부속기관침범이 1118

5 - 김보람외 : 육아종증다발혈관염의후향적분석 - Table 3. The characteristics of patients with granulomatosis with polyangiitis at the time of the diagnosis and the ophthalmic clinic visit Patient No. Ophthalmic manifestation GPA Diagnosis date (symptom) 1 Episcleritis (OU) Apr (ARDS Lab review & Renal biopsy) First visit date (Ophthalmology) & GPA activity Feb , Remission after ocular symptom Unknown (transfer out) Last Post f/u lab f/u period (months) 58 2 Scleritis (OU) 1999 (FUO Renal biopsy, TBLB) Aug , Remission Death c-anca (26.0), p-anca (172.0), 60 3 Retinal vasculitis (OD) Peripheral keratitis (OD) 1993 (dyspnea, sputum Abnormal Chest X-ray, ANCA+) Sep , Death c-anca (negative), Orbital inflammation (OS) May , First diagnosis No c-anca (equivocal), 19 5 Dacryoadenitis (OU) 2003 (nasal obstruction Nasal septum biopsy, ANCA+) Nov , Remission Unknown (transfer out) c-anca (negative), 72 6 Orbital inflammation (OU) NLD obstruction (OU) 1998 (Nasal septum perforation symptom & Lab review) Aug , Remission Death c-anca (141.0), p-anca (31.0), 72 7 NLD obstruction (OU) Aug (sinusitis biopsy) Jan , No c-anca (8.0), p-anca (negative), 72 8 NLD obstruction (OU) Orbital cellulitis Mar , First diagnosis Yes c-anca (15.0), 18 9 Orbital abscess (OD) Jun (Orbital pain biopsy by ENT & symptom, Lab review) Jun , Unknown (f/u loss) 30 GPA = granulomatosis with polyangiitis; f/u = follow up; OU = oculus unitas; OD = oculus dexter; OS = oculus sinister; ARDS = acute respiratory distress syndrome; = C reactive protein; FUO = fever of unknown origin; TBLB = transbronchial lung biopsy; c-anca = cytoplasmic antineutrophil cytoplasmic antibody; p-anca = perinuclear antineutrophil cytoplasmic antibody; NLD = nasolacrimal duct; ENT = ear nose throat. 일어난것을확인할수있었다. 망막혹은맥락막에침범하는경우는약 2.9% 정도로매우드물게보고되고있으며, 망막염, 혈관염혹은망막혈관폐쇄등의형태로나타난다. 17 본연구에서는비문증상으로내원한환자 1명 (2%) 에서망막혈관염을진단하였으며, 또한이환자는주변부위각막염또한동반하고있었다. 이외포도막염, 시신경염등의증상으로눈및눈부속기관침범이나타날수있으나, 본연구에서는발견되지않았다. 이전발표된논문들에의하면약 8-16% 환자들에있어서진단당시눈및부속기관의침범을보였는데, 본연구에서는총 2개 (4%) 의증례에서눈및부속기관의이상증상을통해육아종증다발혈관염을진단하였으며, 총 3개 (6%) 의증례에서눈및부속기관의이상증상을통해육아종증다발혈관염의재발을알수있었다. 본연구에서는육아종증다발혈관염안침범률이서양인을대상으로한 연구에비하여전반적으로낮은것을확인할수있었으며, 법적실명에이른환자또한 0명 (0%) 으로이전연구에서의 8% 에비해낮음을알수있었다. 9 육아종증다발혈관염이눈및부속기관으로침범한경우, 일반적으로전신치료에동반되는스테로이드및사이클로포스파미드등의면역억제제등이함께사용되고있으나상공막염이나전방포도막염의경우, 국소적비스테로이드성소염제및스테로이드점안제만으로도치료가되기도한다. 전신적으로는비활동성을보이는단안포도막염과비괴사성공막염의경우국소스테로이드주사술이고려되기도한다. 한편, 프랑스에서진행된 760명의육아종증다발혈관염환자를대상으로한연구결과에따르면유럽인의비율은 90.7% 였던반면북부아프리카인의비율은 4.3% 로유럽인이더많이차지하였고, 이중각각의인종에서눈및 1119

- 대한안과학회지 2017 년제 58 권제 10 호 - A B C D Figure 1. Case 7. This 68-year-old woman presented with epiphora. She had a history of sinusitis and periorbital inflammation.

(C, D) The coronal and axial views of contrast computed tomography show chronic inflammatory changes in form of the destruction of the nasal septum and ethmoid sinus (yellow arrow).

20 하지만현재까지육아종증다발혈관염의눈및부속기관침범에대해아시아인을대상으로한연구는서양에비해많이이루어지지않고있다. 이연구의제한점으로는후향적인연구로육아종증다발혈관염은드문질환으로대상환자수가적고, 눈및눈부속기관의이상증상이있던시점이아닌외래를방문한시점을기점으로가정하였다는점이있다.

또한, 안구증상이육아종증다발혈관염증상의첫발현인경우는상대적으로흔하지않으나, 높은비율로눈및눈부속기관침범이일어나고, 조기진단및치료가중요하므로원인을알수없는결막염, 상공막염, 각막염, 포도막염, 망막혈관염, 비루관폐쇄등의증상이있고, 영상검사를통해안와침범이의심되는병변을발견한경우, 필히전신적증상에대한적절한병력청취, 신체검진과 c-anca, p-anca

6 - 대한안과학회지 2017 년제 58 권제 10 호 - A B C D Figure 1. Case 7. This 68-year-old woman presented with epiphora. She had a history of sinusitis and periorbital inflammation. (A) A photograph showing the collapse of the nasal bridge, also known as the 'saddle nose'. (B) An endoscopic view of the right nasal cavity shows friable mucosa and easy bleeding. (C, D) The coronal and axial views of contrast computed tomography show chronic inflammatory changes in form of the destruction of the nasal septum and ethmoid sinus (yellow arrow). 부속기관침범을보인환자는유럽인에서약 32%, 북부아프리카인은 15% 로유럽인에서더높은비율이었다. 18 또한, 일본에서진행된 241명의육아종증다발혈관염환자를대상으로한연구에서는 46.1% 의환자에서점막과눈관련한증상이있었으며, 19 22명의환자를대상으로한또다른연구에서는 45.5% 의환자에서진단당시안과적침범이있었다. 20 하지만현재까지육아종증다발혈관염의눈및부속기관침범에대해아시아인을대상으로한연구는서양에비해많이이루어지지않고있다. 이연구의제한점으로는후향적인연구로육아종증다발혈관염은드문질환으로대상환자수가적고, 눈및눈부속기관의이상증상이있던시점이아닌외래를방문한시점을기점으로가정하였다는점이있다. 또한단일기관연구로실제적으로눈및눈부속기관의이상증상이발현이되었음에도불구하고본원이아닌타원에내원한환자들은연구에포함되지않았다는점을들수있다. 하지만이제까지한국인을대상으로한육아종증다발혈관염의환자들의안과적임상양상에대하여분석한최초의연구로육아종증다발혈관염의눈및부속기관의침범경향성을파악한데에그의의가있다. 또한, 안구증상이육아종증다발혈관염증상의첫발현인경우는상대적으로흔하지않으나, 높은비율로눈및눈부속기관침범이일어나고, 조기진단및치료가중요하므로원인을알수없는결막염, 상공막염, 각막염, 포도막염, 망막혈관염, 비루관폐쇄등의증상이있고, 영상검사를통해안와침범이의심되는병변을발견한경우, 필히전신적증상에대한적절한병력청취, 신체검진과 c-anca, p-anca 등의검사를통해육아종증다발혈관염의가능성을염두에두어야한다. 육아종증다발혈관염이눈및눈부속기관의이상증상을일으키는경우, 그레이브씨병, 감염및염증성질환등과감별하기는매우어렵다. 따라서전신적인임상양상, 갑상선기능검사, 항체검사, 세균배양검사등을통해이를감별하도록하여야한다. REFERENCES 1) Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. Arthritis Rheum 2011;63: ) Fahey JL, Leonard E, Churg J, Godman G. Wegener's granulomatosis. Am J Med 1954;17: ) Leavitt RY, Fauci AS, Bloch DA, et al. The American College of 1120

7 - 김보람외 : 육아종증다발혈관염의후향적분석 - Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990;33: ) Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994;37: ) Savige J, Dimech W, Fritzler M, et al. Addendum to the International Consensus Statement on testing and reporting of antineutrophil cytoplasmic antibodies. Quality control guidelines, comments, and recommendations for testing in other autoimmune diseases. Am J Clin Pathol 2003;120: ) Rao JK, Weinberger M, Oddone EZ, et al. The role of antineutrophil cytoplasmic antibody (c-anca) testing in the diagnosis of Wegener granulomatosis: a literature review and meta-analysis. Ann Intern Med 1995;123: ) Harper SL, Letko E, Samson CM, et al. Wegener s granulomatosis: the relationship between ocular and systemic disease. J Rheumatol 2001;28: ) Reinhold-Keller E, Beuge N, Latza U, et al. An interdisciplinary approach to the care of patients with Wegener's granulomatosis: long-term outcome in 155 patients. Arthritis Rheum 2000;43: ) Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992;116: ) Joshi L, Tanna A, McAdoo SP, et al. Long-term outcomes of rituximab therapy in ocular granulomatosis with polyangiitis: impact on localized and nonlocalized disease. Ophthalmology 2015;122: ) You C, Ma L, Lasave AF, Foster CS. Rituximab induction and maintenance treatment in patients with scleritis and granulomatosis with polyangiitis (Wegener's). Ocul Immunol Inflamm 2017 Jun 19:1-8. doi: / [Epub ahead of print] 12) Hellmich B, Flossmann O, Gross WL, et al. EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis. Ann Rheum Dis 2007;66: ) Hogan PC, O Connell RM, Scollard S, et al. Biomarkers predict relapse in granulomatosis with polyangiitis. J Biomark 2014;2014: ) Lee SJ, Kim SS, Kim WS. A case of Wegener's granulomatosis with retinal pigment epithelial detachment and subretinal hemorrhage. J Korean Ophthalmol Soc 2005;46: ) Choi JS, Choi KY. Five cases of Wegener's granulomatosis with ocular manifestations. J Korean Ophthalmol Soc 1986;27: ) Jabs DA, Mudun A, Dunn JP, Marsh MJ. Episcleritis and scleritis: clinical features and treatment results. Am J Ophthalmol 2000; 130: ) Bullen CL, Liesegang TJ, McDonald TJ, DeRemee RA. Ocular complications of Wegener s granulomatosis. Ophthalmology 1983; 90: ) Terrier B, Dechartres A, Deligny C, et al. Granulomatosis with polyangiitis according to geographic origin and ethnicity: clinical-biological presentation and outcome in a French population. Rheumatology 2017;56: ) Sugiyama K, Sada KE, Kurosawa M, et al. Current status of the treatment of microscopic polyangiitis and granulomatosis with polyangiitis in Japan. Clin Exp Nephrol 2013;17: ) Tsuchida Y, Shibuya M, Shoda H, et al. Characteristics of granulomatosis with polyangiitis patients in Japan. Mod Rheumatol 2015;25: = 국문초록 = 육아종증다발혈관염의눈및눈부속기관침범에대한후향적분석 목적 : 3 차단일의료기관에내원한육아종증다발혈관염환자들중눈및눈부속기관침범이있던환자들을대상으로치료및경과를분석하여보고한다. 대상과방법 : 2008 년 7 월부터 2014 년 6 월까지 3 차단일의료기관에내원한환자중육아종증다발혈관염으로진단된환자들에대한의무기록을후향적으로분석하였다. 결과 : 해당기간동안연구에참여한단일의료기관을방문한육아종증다발혈관염환자는총 51 명이고이들중안과외래에내원한환자는 21 명이었다. 이들중 9 명이눈및눈부속기관의이상증상이있었으며 ( 남자 4 명, 여자 5 명 ), 이는상공막염 (2%), 공막염 (2%), 주변부각막염 (2%), 염증성안와질환 (6%), 안와농양 (2%), 망막혈관염 (2%), 비루관폐쇄 (6%) 등의양상을보였다. 이들은임상양상에따라국소스테로이드, 경구스테로이드, 경정맥스테로이드요법및사이클로포스파미드등의면역억제제치료를받았으며, 비루관폐쇄및농양에대해서는수술적치료가동반되기도하였다. 58 ± 30 개월의경과관찰기간동안모든환자에서치료후임상적호전을보였다. 결론 : 육아종증다발혈관염은비교적드문질환이나종종눈및눈부속기관침범소견을보이며, 실명까지진행될수있기에이에대한적절한진단과치료및경과관찰이필요하다. 하지만, 이에대해아시아인을대상으로한연구는서양에비해많이이루어지지않고있으며, 본연구에서는질병의눈및눈부속기관침범이기존에발표된논문들에비해적은것으로나타났다. < 대한안과학회지 2017;58(10): > 1121

<283732372D3733312920B4D9C3CAC1A120BCD2C7C1C6AEC4DCC5C3C6AEB7BBC1EEC0C720B3EBBEC8C0C720BDC3B7C2BAB8C1A4BFA120B4EBC7D120C0AFBFEBBCBA20C6F2B0A1283035292E687770>

<283732372D3733312920B4D9C3CAC1A120BCD2C7C1C6AEC4DCC5C3C6AEB7BBC1EEC0C720B3EBBEC8C0C720BDC3B7C2BAB8C1A4BFA120B4EBC7D120C0AFBFEBBCBA20C6F2B0A1283035292E687770> 대한안과학회지 제 49 권 제 5 호 2008 J Korean Ophthalmol Soc 49(5):727-731, 2008 DOI : 10.3341/jkos.2008.49.5.727 다초점 소프트콘택트렌즈의 노안의 시력보정에 대한 유용성 평가 김현경 1 김효명 2 정성근 1 가톨릭대학교 의과대학 성모병원 안과학교실 1, 고려대학교 의과대학 안암병원 안과학교실