서준벌외 : 며 혈관염 중성구, 럼프구, 형질세포 (p lasma cell), 조직구 (histiocyte) 및거대세포 (giant cell) 의침윤을통반한괴사성육아종성혈관염을보인다 (1). 기관이나기관지를침범한경우에는벽의비후나기관, 기관지내의종괴로보이며폐허탈에의한소견

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1 대한발사선의학호 지 1999 ; 40 : 263-2?3 폐혈관염 : 영상소견 1 서준범 엄청기 정진욱 송재우 2 구진모 박재형 연경모 혈관염은혈관을침범하는염증성과정으로결국혈관을파괴하고관련된조직의허혈성손상을야기한다. 폐는혈관염이잘발생하는장기이다. 지금까지폐혈관염을분류하려는다양한노력이있었으나그임상소견및병리소견의상당한중복에의하여공통적인동의에이르지는못하였다. 저자들은폐혈관염을크게육야종성혈관염 (angii tis -gr anulomat osis) 군, 모세혈관염에의하여미만성폐출혈로나타나는군, 그리고그외의다양한혈관염군으로냐누고, 그특정적인흉부X선및 C T소견을각질환의간단한임상상과함께보여주고자한다. 혈관염은혈관을침범하는염증성과정으로혈관을파괴하고 관련된조직의허혈성손상을야기한다. 특히폐는혈관염이잘 발생하는장기인데그이유는광범위한혈관망 ( r ich vascular network) 을가지고있으며기관을통하여많은감작항원 (sensitizing antige n ) 이폐에도달하고폐내에는많은혈관에 작용히는세포 ( vasoacti ve cells) 를가지고있기때문이다 (1). 많은질환이폐혈관염을유발하며다양한분류법이제시되고 있으나공통적인동의에이르지는못하였으며일부에서는여러 질환이중복되는경우도있다 (1, 2). 여러가지다른질환에도 불구하고, 혈관염은공통적인면역병인 (immunopathogenesi - s) 을가지고있는것으로보인다. 폐혈관염의임상상이나방사 선학적인소견은혈관벽의염증이나동반된폐실질의염증등 과연관된경우가많다 (3). 폐혈관염의진단은쉽지않아서임 상소견, 검사소견, 그리고영상소견을종합하여이루어지는경 우가많으며진단의기준은조직생검이다. 저자들은폐혈관염을크게육아종성혈관염 (ngiitis -granu lomatosis) 군, 모세혈관엽과더불어미만성폐출혈로나타나 는군, 그리고그외의다양한혈관염군으로나누고그방사선 학적인소견을보여주고자한다. 육아종성혈관염군 (ngiitis - granulomatosis group) 이질환군은전통적으로베게너육아종증 (W e ge ner s granulomatosis), 알레르기성혈관염및육아종증 (a llergic angiitis and granulomatosis, Churg-Strauss syndrome), 럼프종양육 아종증 (l y mphomatoid granulomatosis ), 괴사성사르코이 l 서울대학교의과대학방사선과학교실, 방사선의학연구소 2 서울시럽보라매병원진단방사선과이논문은 1998 년 7 월 20 일접수하여 년 9 월 28 일에채택되었음. 드육아종증 (nec r ot i z ingsarcoi d granulomatosis), 기관지중심성육아종증 (b r on chocent ri c granulomatosis) 등이포함되어있다 ( 4). 이중진정한전신성혈관염은베게너육아종증, 알레르기성혈관염및육아종증이며, 럼프종양육아종증은혈관침범성을보이는악성종양으로구분하는경향이있으며, 괴사성사르코이드육아종증은혈관염을동반한사르코이드증의변이로생각하기도한다. 기관지중심성육아종증은혈관중심성 (angio cen tric) 을보이는다른질환과는달리기관지중심성 (bronchocentr ic) 분포를보이며일차적인혈관염이라기보다는기관지중심성육아종증의주변혈관침범으로생각된다 (4). 각질환이비슷한방사선학적소견과임상상을보이지만약간의차이가있다 (Tab le 1). 베게너육아종증 (Wegene r's granulomatos;s) 이질환은상, 하부호홉기관과신장등을침범하는비교적잘알려진혈관염증후군이다 (1-4). 일부에서는신장을침범하지않으며호홉기만을침범하는제한형 (limi ted form ) 이보고되고있다. 남녀비는같으며모든나이에서발생할수있으나평균연령은 40셰이다. 폐침윤은질병의진단탕시가장흔히보이는소견이다. 전형적인폐소견은수 mm에서 10cm에이르는 원형의분명한경계를가지는결절이나폐침윤으로, 대개다수이며양측성이다 (2-4) % 정도에서공동화 (cavi - tation) 가보이는데공동은두터운벽을가지고있으며내부는불규칙한경우가많다 (F i g. 1). 공동벽의두께는병변이진행함에따라앓아져서결국매우앓은벽을갖는기냥 ( thin-walled air cy st) 으로보이기도한다 ( 2-4). CT에서도마찬가지로다수의결절로나타나는경우가많으며, 흉막과인접한쐐기모양의음영이보일수있고 (Fig. 2), 결절내로공기 -기관지음영 (air -bronchogram) 이보이기도한다. 병리학적으로폐결절은 %

서준벌외 : 며 혈관염 중성구, 럼프구, 형질세포 (p lasma cell), 조직구 (histiocyte) 및거대세포 (giant cell) 의침윤을통반한괴사성육아종성혈관염을보인다 (1). 기관이나기관지를침범한경우에는벽의비후나기관, 기관지내의종괴로보이며폐허탈에의한소견을동반할수있고 CT에서침범벙위와양상을정확히판단할수있다 (2) (Fig. 2).

2 서준벌외 : 며 혈관염 중성구, 럼프구, 형질세포 (p lasma cell), 조직구 (histiocyte) 및거대세포 (giant cell) 의침윤을통반한괴사성육아종성혈관염을보인다 (1). 기관이나기관지를침범한경우에는벽의비후나기관, 기관지내의종괴로보이며폐허탈에의한소견을동반할수있고 CT에서침범벙위와양상을정확히판단할수있다 (2) (Fig. 2). 흉막삼출 (p leura l effusion) 은 20-50% 정도로다양하게보고되고있으며, 소동맥, 모세혈관, 소정맥염을일으킨경우에는미만성폐출혈로나타날수있다 (2-4). 그외 에드문소견으로기관지흉루 (bronchopleural fistula), 공동내진균종 (mycetoma) 형성, 기흉등이보고되고있다 ( 4). 엄상검사에서혈청및기관지폐포세척액 (bronchoalveoalr lavage fluid) 내의항중성구세포질항체 (antineutrophil cytoplasmic antibody, NC) 는매우높은진단의정확도를보이며 (90 % 이상 ), 질병의활성도 (activity) 와도밀접한관련이있다고한다 (2-3). 과거에는치명적인질환이었으나, 스테로이드와 cyclophosphamide의사용으로현재는예후가좋아서 90% 정 Table 1. Pu1monary ngiitis and Granulomatosis : Radiological Comparison (from reference 4) Wegener s Lymphomatoid Necrotizing Sarcoid ronchocentric Granulomatosis Granulomatosis Granulomatosis Granulomatosis Pattern Multiple nodules Multiple nodules Multiple nodules Solitary nod ule Consolidation less Consolidation, Consolidation frequent Interstitial less less frequent frequent Changing pattern Changing pattern Interstitial frequent Distribution ilateral ilateral ilateral Unilateral Diffuse Mid- and lower Diffuse Upper lung lung Cavitation Common Less common Occurs Infrequent denopathy Rare Infrequent Uncommon Infrequent Pleural effusion Infrequent Uncommon Uncommon Infrequent llergic ngiitis and Granulomatosis Nonspecific infiltrates Nodules nywhere Rare Rare Infrequent ~ Fig year-old man with Wegener's granulomatosis presenting with abnormal chest radiograph and hematuria. Renal and skin biopsy confirmed the diagnosis of Wegener s granulomatosis. The titer of serum NC was elevated, which is characteristic laboratory finding of Wegener's granulomatosis. Chest radiograph shows about 4-cm sized mass with central irregular air-cavity (arrow) in left apex. nother well-marginated nodule is seen in right lower lobe(white arrow). Chest tube in left hemithorax is due to post-biopsy pneumothorax. 8. CT scan at the level of the lung apex shows thick-walled cavitary mass(arrow), which has irregular inner margin. Note peripheral rim enhancement suggesting area of inf1lammatory hypervascularity, in contrast to low-attenuated wall of the cavity(star), suggesting necrotic portion. 없

대한밤시선의학회지 1999; 40: 263-273 도가완전관해 (complete remission) 에이른다고한다 0-4). 치료중간이나재발시동반된감염이잘알려진합병증이며대 개의경우포도싱구균 (Stathylococcus aureus) 에의한 1 감염이 다. 결절의크기가증가하고공동내에공기액체층 (air f1uid leve I) 을보이면감염을의심하여야한다.

3 대한밤시선의학회지 1999; 40: 도가완전관해 (complete remission) 에이른다고한다 0-4). 치료중간이나재발시동반된감염이잘알려진합병증이며대 개의경우포도싱구균 (Stathylococcus aureus) 에의한 1 감염이 다. 결절의크기가증가하고공동내에공기액체층 (air f1uid leve I) 을보이면감염을의심하여야한다. 이경우항생제치료 에는잘반응하지않으며수술적치료를펼요로한다 (1, 4). Fig year-old man with Wegener s granulomatosis who has been treated with anti-tuberculous chemotherapy for 4 months. Repeated sputu띠 examinations for I F were negative. Renal biopsy revealed crescentic glomerulonephritis, consistent with Wegener s granulomatosis. l?espite of intensive treatment with steroid and cyclophosphamide, he expired.. CT scan obtained after intravenous injection?f contrast media shows large irregular consolidation in right upper lobe and pleural based consolidation in the supe ~ ior segment of right lower lobe. Central low-attenuation area (white arrow) containing irregular air-filled cavity is no뼈 within the consolidated lung suggesting area of neerosis. Note irregular thickening of distal portion of right main :bronchus(arrowheads), which is bronchial involvement of Wegener s granulomatosis. nother nodule is seen in left lung(white open arrow) CT scan obtained at the level of the lung base fhows multiple nodules and consolidanons With or Wlthout cavitation in both lungs(arrows). Fig year-old female with Churg-Strauss Isyndrome presented with generalized skin lesions for 9 months and bronchial asthma for 5 years.. Chest radiograph shows multifocal patchy corlsolidation in both middle and lower lungs. Cardiomegaly was due to pericardial effusion, which is cardiac manifestation of Churg-Strauss syndrome. High-resolution CT scan demonstrates linear atelectasis and consolidation in both Iower Iobes (arrows) with surrounding ground-glass opacity. lood eosinophilia was documented and the result of skin biopsy was consistent with Churg-Strauss syndrome. Mononeuritis multiplex Ideveloped 4 months Iater

서준범오 I : IHI 휠관염 알레르기성혈관염및육아종종 (llergic angiitis and granulomatosis, Churg-Strauss syndrome) 이질환은천식, 말초혈관호산구증과더불어폐, 피부, 신경등을침범하는전신성혈관염을특정으로하는절환으로신장침범이가장혼하다 (1-4). 주로중년에발병하며남녀비는같다.

4 서준범오 I : IHI 휠관염 알레르기성혈관염및육아종종 (llergic angiitis and granulomatosis, Churg-Strauss syndrome) 이질환은천식, 말초혈관호산구증과더불어폐, 피부, 신경등을침범하는전신성혈관염을특정으로하는절환으로신장침범이가장혼하다 (1-4). 주로중년에발병하며남녀비는같다. 폐혈관염은병리학적으로주로중간크기의동, 정맥을침범하는혈관엽과호산구침윤, 괴사성의혈관외육아종형성을특정으로한다 (1). 약 75% 의환자에서폐침범이발견된다. 방사선학적소견은다수의비분절성폐첨윤으로대부분주변부에위치한다 (Fig. 3). 그외에다수의결절로보일수도있으며 (Fig. 4), 병소 7} 이동동 } 는양상을보이기도한다 (2-4). 공동 형성은비교적드물다. 폐침윤이대칭성인경우만성호산구성폐렴처럼보이기도한다 (2). 흉막삼출은 30% 정도에서보인다 (Fig. 3). 그외에드물게폐문부럼프절종대, 미만성간질성음영, 속렵성결절 ( miliary nodule) 로보일수있다 (4). 심장을침범하는경우, 심장비대, 심낭삼출등을보이기도한다 (Fig. 3). 상부기도를침범하는경우는드물다. 베게너육아종증과마찬가지로스테로이드나세포독성약물 (cytotoxic agen-t) 에잘반응한다 (1 ). 진단을위해서는혈관염을증명하여야하는데, 비슷한소견을보일수있는특발성호산구증가증 (i diopat hic hypereosinophilic syndrome), 호산구성폐렴등을배제하기위한것이다 (1). 림프종앙육아총증 (Lymphomatoid granu/omatosis) 괴사및혈관주변의침윤을통반하는혈관중심성의럼프세 망세포의증식을특정으로동 } 는드문질환으로폐이외에피부, 신경계를침범한다. 남자에서조금더많이발생하며평균연령은 50세이다. 병리학적으로럼프구, 형질세포, 조직구및비전형적인럼프세망세포의미만성증식을특정으로동 } 는다발성의결절이며소혈관벽의침윤과국소적괴사를흔히동반한다. 방사선학적으로는다양한크기의다발성결절빛폐포성침윤이양측폐의주로하엽에분포하는데괴사에의한내부저음영이나공동형성을볼수있다 (4) (Fig. 5). 이러한소견은베게너육아종증과는구별되지않는소견이다. 그러나베게너육아종증과는달리상부기도침범은드물다. 흉막삼출은 33% 에서동반되며, 종격동몇폐문부럼프절종대는드물고럼프절종대를동반한경우는렴프종으로진행을의심하여야한다 (4). 이질환은과거에는일차성폐혈관염으로생각되어왔으나최근악성럼프종으로의전이 ( transformation) 등을바탕으로혈관염이라기보다는악성렴프종의변이로생각되는경향이있다 (1 ). 괴사성사르코이드육아종증 (Necrotizing sarcoid granu/omatosis) 괴사성혈관염과혈관주위의사르코이드성육아종형성을특정으로하는질환으로전형적인사르코이드증과는탈리폐외의침범이드물며, 폐문부, 종격동럼프절종대가적다 ( 4). 방사선학적인소견은다수의양측성결절로보이는경우가많으며 (Fig. 6), 1/ 4에서는단일종괴로보이기도한다. 공동형성은 드물다 (4). 흉막침범은연구에따라다양하여일부에서는드물다고하는반면, 일부에서는흉막침범이전형적인사르코이드 1... Fig year-old woman with Churg-Strauss syndrome presented with abnormal chest radiograph and known bronchial asthma for two years.. Chest radiograph shows multiple ill-defined nodular opacities in both lower lungs(arrows).. High-resolution CT scan at the level of lung base demonstrates multiple nodular lesions with irregular border and peripheral ground-glass opacification. Skin biopsy for erythematous lesion revealed necrotizing vasculitis with eosinophilic infiltration, consistent with Churg-Strauss syndrome. 짜ω

대한방사선의학회지 1999; 40: 263-273 증과의다른점이라고주장하고있다 (2). 기관지중심성육아종증 (ronchocentric granulomatosis) 이질환이다른육아종증과다른점은이름에서알수있듯이병리학적으로혈관중심성이아난기관지중심성분포를보인다는것이다. 이질환은하나의질병이라기보다는다양한병인에대한특정적, 공통적인병리반응이라고보는견해도있다 (3).

5 대한방사선의학회지 1999; 40: 증과의다른점이라고주장하고있다 (2). 기관지중심성육아종증 (ronchocentric granulomatosis) 이질환이다른육아종증과다른점은이름에서알수있듯이병리학적으로혈관중심성이아난기관지중심성분포를보인다는것이다. 이질환은하나의질병이라기보다는다양한병인에대한특정적, 공통적인병리반응이라고보는견해도있다 (3). 병리적으로기관지중심성괴사성육아종성염증을특정으로한다. Katzenstein은기관지중심성육아종증을크게천식군과비천식군으로나누어서분류하였는데천식군은주로 20대의남자에서많으며칸디다 (candid a) 나아스페르질루스 (aspergillus) 진균에대한혈청검사가양성인경우가많고, 실제괴사성기관지, 세기관지조직내에비침습적인진균 (fungal hyphae) 이발견되는점등을고려할때진균감염에대한과민성반응 (hypersensitivity reaction) 이라고주장하였으며, 엄상적, 방사선학적으로도알레르기성기관지폐아스페르질루스증 (a llergic bronchopulmonary aspergillosis, P) 의소견을보이는경우가많다. 반면에비천식군의경우에는주로 50대의여자에서많으며다른항원에대한과민성반응으로생각된다 (3, 4). 방사선학적으로는주로결절이나기강경결로보이는데일측성이며폐상부를주로침범한다 (Fig. 7). 드물게알레르기성기관지폐아스페르질루스증과같은기관지확장증이나점액충만 (mucoid impaction) 에의한가지치는모양의음영을보이기도하며미만성망상결절 (reticulonodular) 음영을보일수있다 (Fig. 7) (4). 모세혈관염에동반된미만성폐출혈 (Diffuse pulmonary hemorrhage with capillaritis) 미만성폐출혈은폐내의미세혈관에서생기는광범위한출혈 에의한증후군으로객혈, 빈혈, 흉부 X 선에서기강경결을특정 으로한다 (5). 병리학적으로는폐포내와폐간질내에최근의 출혈과, 헤모시데린을포함한대식구 (hemosiderin-laden macrophage) 를볼수있다 (5). 원인에상관없이흉부 X 선소견은 일정한데, 급성기에는국소적이거나융합성의 (confluent) 기강 음영이특정이며, 이러한음영은주로폐문부주위에분포하거 나미만성얼수있다 (2, 5) (Fig. 8, 9). 대개폐주변부나폐첨부 는침범되지않는다. 양측성인경우가많으며기관지 - 공기음 영을흔히동반한다. 이러한소견은비특이적이어서감염이나 폐부종과구별되지않는다.2-3 일내로폐포성음영은소실되 며망상의음영을보이게된다. 재발하지않는경우는 1-2 주 내에정상화되지만재발하는경우에는간질성음영으로바뀐 후폐섬유화로진행하게된다 (2, 5). 급성기에광범위한폐출혈 이있음에도불구하고정상흉부 X 션소견을보이기도한다 (2, 5). 이경우 CT 를시행하면거의모든예에서폐실질의이상을 볼수있다. CT 에서급성기에는주로기장정결이나간유리음 영을보이며 (Fig. 8, 9) 아급성기에는 1에서 3mm 크기의무수 한결절을보언다 (2). 미만성폐출혈은국소적폐출혈과이에 동반한혈액흡인과구별하여야한다. 국소적폐출혈은주로만 성기관지염이나기관지확장증, 종양, 염증등에동반된다. 기관 지폐포세척액에서많은양의헤모시데련을포함한대식구를 Fig year-old man with lymphomatoid granulomatosis presented with general weakness. Contrast enhanced CT scan shows irregular nodular consolidations along the bronchovascular bundles. The central portion of lesions shows low attenuation (arrowheads) due to necrosis. Note also small nodular lesion in left lung(white arrow). No hilar or mediastinal lymph node enlargement is noted. Open lung biopsy revealed lymphomatoid granulomatosis. [ / χω... Fig year-old female with necrotizing sarcoid granulomatosis presented with cough and dyspnea. There were multiple nodules scattered in both lungs on chest radiograph(not shown). CT scan obtained at lung base shows multiple nodular lesions in right lower lobe(arrows). Cavitation of the nod ule was noted in right upper lobe(not shown here). Open lung biopsy revealed necrotizing sarcoid granulomatosis F이 low-up CT scan obtained after steroid treatment for one month showed markedly decreased size of the nodules(not shown).

서준범오 1: 며 혈관염 ~ Fig. 7. 38-year-old non-asthmatic patient with bronchocentric granulomatosis presenting with cough and dyspnea.

6 서준범오 1: 며 혈관염 ~ Fig year-old non-asthmatic patient with bronchocentric granulomatosis presenting with cough and dyspnea.. Chest radiograph shows ill-defined mass-like opacity in right middle lung zone(white arrow). Multiple reticulo-nodular opacites in both lower lungs are also noted.. CT scan obtained at subcarinal level shows conglomeration of branching linear opacities forming an ill-defined nodular opacity, suggesing a bronchial or bronchiolar lesion, in right upper lobe(white arrow). Note also several small nodules(white open arrows). C. CT scan at the level of lung base demonstrates multiple nodules and branching structures in both lungs. rea of ground glass opacity is also noted(arrowhead). ronchiectasis and mucus plugging is noted in basal subsegmental bronchi of right lower lobe(white arrows). Open lung biopsy for right upper lobe lesion revealed bronchocentric granulomatosis Fig year-old known woman with systemic lupus erythematosus with presumed diffuse pulmonary hemorrhage presented with fever and hemoptysis.. Chest radiograph shows patchy opacity in both middle and lower lungs. Small amount of bilateral pleural effusion is also noted.. High-resolution CT scan obtained at subcarinal level shows bilateral ground-glass opacity mainly located in central lung. Small amourit of pericardial effusion was noted(not shown). Differential diagnosis includes pulmonary edema, in fection such as Pneumocystis carinii pneumonia, cytomegaloviral pneumonia, and diffuse pulmonary hemorrhage. The opacity has markedly decreased in extent and severity on follow-up chest radiograph obtained 3 days later and disappeared on chest radiograph obtained 2 weeks later(not shown)

대한방시선의학호 지 1999; 40: 263-273 r- Fig. 9. 59-year-old female with rriicroscopic polyarteritis nodosa presented with sudden hemoptysis.

7 대한방시선의학호 지 1999; 40: r- Fig year-old female with rriicroscopic polyarteritis nodosa presented with sudden hemoptysis.. Chest radiograph shows consolidation in bilateral perihilar and left upper lung zone(arrows). Small amount of left pleural effusion is noted.. CT scan shows multifocal consolidation in both lungs. The consolidation is inhomogeneous and associated areas of ground-glass opacity are due to partial filling of air space. Open lung biopsy revealed hemosiderin laden macrophages in the alveolar space and interstitium with capillaritis, consistent with microscopic polyarteritis nodosa. v / / Fig. 10. Takayasu s arteritis causing multiple aneurysms and beaded appearance of the pulmonary artery in a 26-year-old woman presenting with dyspnea.. Pulmonary arteriogram shows multiple aneurysms of the pulmonary artery(arrowheads). Note beaded appearance of a branch of pulmonary artery in left lower lobe (arrow).. Maximum intensity projection CT angiogram shows beaded aneurysmal dilation of pulmonary artery(white arrowheads). Note also triangular increased attenuation at superior segment of the right lower lobe due to infarction (white arrow) c. CT scan at the level of the right inferior pulmonary vein shows beaded appearance of a branch of the pulmon- ary artery (arrow).and aneurysmal dilatation of pulmonary C artery in left lower lobe(arrowhead). Inhomogenous parenchymal attenuation in both lungs is due to multiple narrowing of pulmonary arteries causing decreased perfusion. %

8 서준벌오 1: 며 혈관염 발견하는경우진단에도움이된다. 모세혈관염을동반하는경우는베게너육아종증, 미세결절성다발동맥염 (microscopic polyarteri tis nodosa), 혼합형한랭글로불린혈증 (mixed cryglobulinemia), 베체트증후군 (ehç et s syndrome), Henoch-Schönlein 자반병, Goodpasture 증후군, 전신흥반성루푸스 (systemic lupus erythromatosis) 등의결체조직질환등이있다 (6). Goodpasture 증후군과전신흥반성루푸스일부에서는모세혈관염을통반하지않는미만성폐출혈이보고되고있다. 베게너육아종증에대해서는전술하였고, 여기서는대표적인결체조직질환언전신홍반성루푸스 (systemic lupus erythromatosis) 과미세결절성다발동맥염 (microscopic polyarteritis nodosa) 를소개한다. 전신홍반성루푸스 (System;c lupus erythematos;s, SLE) 전신홍반성루푸스는결체조직질환중미만성폐출혈을동반할수있는가장흔한질환이다 (5). 전신홍반성루푸스에서감염을제외하고흉막, 폐에생기는합병증으로그빈도에따라흉막염, 흉막삼출, 급성루푸스폐렴을포함한간질성폐렴, 폐 색전증, 미만성폐출혈, 그리고혈관염과동반된일차성폐동액고혈압을들수있다. 미만성폐출혈은드물지만 (2%) 심각한합병증의하나로그치사율이 60-90% 에달한다 (6). 대개미만성폐출혈을일으키는경우이미진단된상태인경우가많으며, 많은경우에흉막, 폐에다른병변을가지고있는경우가많다 (5, 6). 전신홍반성루푸스환자에서미만성의폐음영을보이는경우 (Fig. 8) 감염, 홉인, 울혈성심부전, 신부전등의다른원인과구별하는것이중요하며경우에따라서폐생검이필요할수있다. 미세결절성다발동맥염 (M;croscop;c polyarter;t;s nodosa) 결절성다발통맥염 (polya rteri tis nodosa) 의변형으로생각되는질환으로주로소혈관을침범하는전신성혈관염과국소성사구체신염을특정으로한다 (2, 6). 1/ 3에서폐를침범하여미만성폐출혈을일으키며늑막삼출을동반하기도한다 (1 5%) (Fig. 9) (2). 폐를침범하는경우치명적일수있다. 반면전형 적인결절성다발동맥염은주로폐외조직의중간크기의혈관을침범하며폐출혈을일으키지않는다. Fig. 11. Takayasu s arteritis in active stage in a 26-year-old woman with delayed enhancement of vessel wall on CT scan. xial CT angiogram at the level of the thoracic inlet shows marked narrowing of the lumens of the major branches of the aortic arch and thickening of the vascular wall (arrows).. CT angiogram in arterial phase shows thickening and enhancement of the wall of the aorta and main pulmonary artery(arrows). c. CT scan obtained 60 minutes after shows diffuse enhancement of the vascular wall(arrows). indicating active stage of vasculitis. c

대한발사선의학회지 1999; 40: 263-273 수있게되었다. 특히조영증강후내측의저음영의띠와혈관 그외의폐혈관염타카야수동맥염 (Takayasu s arteritis) 원언을알수없는일차성혈관염으로주로대동맥과그주요분지, 그리고폐혈관을침범한다. 극동아시아에많으나전세계에퍼져있는질환으로주로젊은여자에많다 (2).

9 대한발사선의학회지 1999; 40: 수있게되었다. 특히조영증강후내측의저음영의띠와혈관 그외의폐혈관염타카야수동맥염 (Takayasu s arteritis) 원언을알수없는일차성혈관염으로주로대동맥과그주요분지, 그리고폐혈관을침범한다. 극동아시아에많으나전세계에퍼져있는질환으로주로젊은여자에많다 (2). 폐혈관침범은흔해서약 50% 정도까지보고되고있으나임상증상은없는경우가많다 (1). 병리학적으로대혈관의벽을침범하여섬유화와염증세포의침윤을동반한다. 전형적인혈관조영술소견은섬유화에의한협착, 폐쇄, 그리고동맥류형성등이다 (2). CT 혈관조영술을시행하면고식적혈관조영술에서와마찬가지로혈관내경의변화를볼수있으며혈류감소에따른모자이크양 상의폐실질저음영부위를확인할수있다 (Fig. 10). 최근박 등 (7) 의연구에따라나선식 CT를이용하여 CT 혈관조영술을시행함으로써혈관내경의변화와더불어혈관벽의변화를볼 벽의불규칙한조영증강을보이는경우는혈관벽에활동성염증이진행되고있음을시사하는소견이다 (Fig.11). 베체트증후군 (ehcet s syndrome) 베체트증후군은만성척이며, 재발을잘하는전신성혈관염으로모든크기의혈관을침범할수있다 (2). 흉부에서는상대정맥의혈전증, 폐동맥의혈전에의한폐색에따른폐경색, 폐동맥류, 혈관염에혈관파열이나모세혈관염에따른폐출혈등이생긴다 (2, 8). 흉부X선에서폐출혈, 경색에따른기강경결, 동맥류에의한폐종괴, 상대정맥폐쇄에다른종격동부종에의한종격동확장을볼수있으며, CT에서상대정맥폐쇄협착과종격동부종, 그리고흉벽등에분포한측부혈관 (collateral vessels) 들을볼수있다. 폐병변으로는기강경결, 그리고폐동맥류 를볼수있다 (8) (Fig. 12, 13). 가끔흉막삼출을동반할수있 는데폐경색에따른이차적인소견으로생각된다 (2). Fig. 12. Thoracic ehçet s syndrome in 36-year-old man presenting with hemoptysis and aneurysmal dilatation of the pulmonary artery.. Chest radiograph shows a round mass in the right lower lung.. Contrast enhanced CT scan shows huge dilatation of the right descending pulmonary artery containing intraluminal thrombus(white arrow). Note also the dilated left descending lobar pulmonary artery(white open arrow). C. Contrast enhanced CT scan obtained 6 months before shows thrombosis of the SVC and right brachiocephalic vein (arrow) and increased attenuation of the Illediastinal fat, which is suggestive of edema. Note also the collateral vessels in the mediastinum and chest wall

서준범오1: 며 혈관염 첨습성펴 l 국균증 (lnvas;ve p 비 monaryasperg; I/ os;s) 환자의면역기능이저하되는경우에진균이침습성을가지게 되며, 특히급성백혈병의항암치료기간중에중성구 ( neutrop hi l) 의 심한감소로인하여침습성폐국균증의위험이증가한 다. 가장흔한소견은괴사성기관지폐렴과균의폐혈관침범에 따른출혈성경색이다.

10 서준범오1: 며 혈관염 첨습성펴 l 국균증 (lnvas;ve p 비 monaryasperg; I/ os;s) 환자의면역기능이저하되는경우에진균이침습성을가지게 되며, 특히급성백혈병의항암치료기간중에중성구 ( neutrop hi l) 의 심한감소로인하여침습성폐국균증의위험이증가한 다. 가장흔한소견은괴사성기관지폐렴과균의폐혈관침범에 따른출혈성경색이다. 균이근위부폐동맥을침범뺨경우, 동액의폐쇄를일으키거나가성동맥류를만들수있다 (9). 이 경우조영증강 CT 에서혈관의폐쇄및가성동맥류로의조영제의유출을볼수있다 (Fig. 14). 결핵성가성동맥류 (Rasmussen aneurysm) 결핵성가성동맥류주위의결핵성공동에의한폐동맥벽의미란에의하여생기는가성동맥류로흔하지않는질환이다 (10). 활동성결핵환자에서다량의객혈을보이는경우대부분기관지동맥에의한출혈이많으나, 혈관조영술상기관지동맥이정상이거나, 기관지동맥색전술 (bronchial artery embolization) 후에도객혈이벚지않는경우에는늑간동맥, 쇄골챔맥, 내유선동맥등의다른체성혈관 (systemic vessel) 과더불 一... Fig. 13. Thoracic ehçet s syndrome in 36-year-old man presented with hemoptysis due to pulmonary infarction. CT scan shows pleural based opacity in right lower lobe, suggesting pulmonary infarction(arrow). Open lung biopsy revealed vasculitis and acute hemorrhagic infarctlon. Fig year-old man with acute myelogenous leukemia complicated by invasive aspergillosis presenting with hemoptysis caused by invasion of the right pulmonary a~tery. Contrast enhanced CT scan shows airspace consolidation in right lower lobe containing round collection of extra-vasated contrast media (white arrow) due to rupture of right descending pulmonary artery. The pat ient died with an extensive pulmonary hemorrhage just after the CT scan. 8 Fig year-old man with Rasmussen aneurysm presented with massive hemoptysis.. Contrast enhanced CT scan shows consolidation with air cavities in left upper lobe. Note round contrast media filling space within the consolidated lung(arrow). Sagittal reformatted CT image shows round contrast media filling lesion, indicating a pseudoaneurysmal sac, surrounded by low-attenuated rim suggesting hematoma(arrow). Note adjacent branch of pulmonary artery (arrowhead) which is thought to be a feeder of this pseudoaneurysm. 낀

11 대한밤시선의학호띠 11999;40: 어폐동맥가성동맥류에의한출혈의가능성을고려하여야한 다 (1 0). CT 에서결핵에의한폐병변과더불어가성동맥류내로 의조영제의유출을볼수있다 (Fig. 15). :i<~ τ1 드프 등4 口 1. Leavitt RY, Fauci S. Pulmonary vasculitis. m Rev Respir Dis 1986; 134: mstrong p, Wilson G, Dee p, Hansell DM. Imaging of diseases of the chest. 2nd ed. St. louis:mosby-year book, , Fraser RS, Pare JP. Synopsis of disease of the lung. 2nd ed Philadelpia : Saunders, 1994: Weisbrod GL. Pulmonary angitiis and granulomatosis: a review J Can ssoc Radiol 1989; 40: Müller NL, Miller RR. Diffuse pulmonary hemorrhage. Radiol Clin North m 1991; 29: Schwarz MI. Cherniack RM, King TE. D 얘 use alveolar hemorrhage and other rare infiltrative disorders. In Murray JF, Nadel J. Textbook of respiratoη medicine. Philadelphia: Saunders, 1994: Park JH, Chung JW, 1m J-G, Kim SK. Park Y, Han MC. Takayasu arteritis : evaluation of mural changes in the aorta and pulmonary artery with CT angiography. Radiology 1995; hn JM, 1m J-G, Ryoo JW, et al. Thoracic manifestation of ehçet syndrome: radiographic and CT findings in nine patients. Radiology 1995; 194: Hayashi H, Takagi R, Onda M, Kumazaki T. Invasive pulmonary aspergillosis occluding the descending aorta and left main pulmonary artery: CT features. J Comput ssist Tomogr 1994; 18: Santelli ED, Katz DS, Goldschmidt M, Thomas H. Embolization of multiple Rassmussen aneurysm as a treatment of hemoptysis. Radiology 1994; J Korean Radiol Soc 1999; 40 : Pulmonary Vasculitis : Imaging Features 1 Joon eom Seo, M.D., Jung-Gi Im, M.D., Jin Wook Chung, M.D. Jae Woo Song, M.D. 2, Jin Mo Goo, M.D., Jae Hyung Park, M.D., Kyung Mo Yeon, M.D. 1 Department 0/ Radiology, and the Institute 0/ Radiation Medicine, Seoul National University College 0/ Medicine 2Department 0/ Radiology, Seoul City oramae Hospital Vasculitis is defined as an inflammatory process involving blood vessels, and can lead to destruction of the vascular wall and ischemic damage to the organs supplied by these vessels. The lung is commonly affected. number of attempts have been made to classify and organize pulmonary vasculitis, but because the clinical manifestations and pathologic features of the condition overlap considerably, these afforts have failed to achieve a consensus. We classified pulmonary vasculitis as belonging to either the angitiis-granulomatosis group, the diffuse pulmonary hemorrhage with capillaritis group, or. other'. Characteristic radiographic and CT findings of the different types of pulmonary vasculitis are illustrated, with a brief discussion of the respective disease entities Index words: Lung, hemorrhage Lung, vascular disease Pulmonary arteries Vasculitis ddress reprint requests to: Jung-Gi 1m, M.D., Depaπment of Radiology, Seoul National University College of Medicine, i 28, Yongon-dong, Chongno-gu, Seoul , Korea Tel Fax 낌

12 1999 년도춘계학술대회사전등록신청서 r--- 연락처 성명전화 : ι 소 -ι --, z 소T 회원구분 : 1) 정회원口 2) 전공의회원口 3) 초빙회원口 우편번호 : 평생회원번호년차 : 전공과목 : 4) 비회원口전공과목 : 학술대회둥록 19æ 년도춘계흑 L 술대회에사전등록을하시겠습니까? 예口 아니오口 예띠를선택하신분은아래에해당하는금액을온라인구좌로입금하십시오. 사전등록 ( 까지 ) : 정회원, 초빙회원, 비회원 50, 000 원전공의회원 20, 000 원 현장등록 ( 이후 ) 정회원, 초빙회원, 비회원 60, 000 원전공의회원 30, 000 원 만 65 세이상원로회원은등록비가면제되나신청서는접수하셔야등록이됩니다, 평생회원이아닌정회원, 초빙회원은년회비 50, 000 원을납입하여야등록이가능합니다. l 온라인송금 해당금액을아래구좌로송금승낸후본신청서의내용을학회 Home Page 에직접입력하시거나본 신청서를우편또는 Fax 로송부하여주시기바랍니다 온라인번호 : 평화은행, 구좌번호 : , 예금주 : 대한방사선의학회 Home Page : http ://radiol. meφkorea.net 학회주소 : 서울시서초구양재동 잉 Te1 (82-2) , Fax (82-2) 소그자셔며 lj " 1 ci 즈는二그 01 o tj e.

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Jksvs019(8-15).hwp Grade I Grade II Grade III 12 대한혈관외과학회지 : 제 20 권 제 1 호 2004 Control Group A Group B Fig. 4. Microscopic findings of vein wall in control, group A and group B on the day of 7 after venous occlusion. The