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2 Fig. 2. Lymphangitic carcinomatosis in a 50-year-old woman. Chest radiograph shows a diffuse increase in interstitial markings in both lungs. However, asymmetry of linear pattern is an important clue to this diagnosis. Known malignancy and failure to respond to diuretic therapy are also helpful. The diagno- sis was confirmed with transbronchial lung biopsy. Fig. 1. Hydrostatic pulmonary edema in a 77- year-old man. Chest radiograph shows diffuse increased interstitial markings and bronchovascular bundle thickening. There are lines radiating from the hilum and lines running perpendicular to the chest wall and these lines are Kerley s A and B lines. Associated cardiomegaly and small pleural effusions are helpful for this diagnosis. 504 Fig. 3. Idiopathic pulmonary fibrosis in a 70-year old-man. Chest radiograph reveals coarse reticular pattern in both lungs, predominantly in basilar and peripheral lung zone. Surgical lung biopsy was not performed and transbronchial biopsy showed nonspecific findings. However, the patient showed all major and minor ATS/ERS criteria for diagnosis of idiopathic pulmonary fibrosis.
3 Fig. 4. Pulmonary fibrosis associated with progressive systemic sclerosis in a 50-year-old woman. Chest radiograph reveals coarse reticular pattern in both lungs and gas-distended esophagus. Peripheral reticular pattern and esophageal dilatation in a woman suggest a diagnosis of progressive systemic sclerosis. Fig. 5. Cystic pattern (left) vs. reticular pattern (right) on chest radiographs. Cystic pattern shows moderate-sized round spaces in the more central lung and reticular pattern shows numerous smaller black holes involving peripheral lung zone. 505 Fig. 6. Lymphangioleiomyomatosis in a 43-year-old woman. Chest radiograph shows multiple moderate-sized fine circular lines in both lungs. Lymphangioleiomyomatosis can be reliably differentiated form Langerhans cell histiocytosis by diffuse lung involvement, infrequency of nodules and more regular shaped cysts.
4 강은영 외: 미만성침윤성폐질환 진단의 영상의학적 접근 한 모양을 보이며, 작은 결절들을 동반하고, 중상부 폐야에 주 로 분포한다(Fig. 7). 유육종증, 규폐증, 탄광부 진폐증 등은 상부 폐야에 더 많이 분포하며(Fig. 9), 반면 패혈증성색전증이나 폐전이는 하부 폐 야에 더 많이 분포한다(Fig. 10). 패혈증성색전증의 결절은 흔 결절음영 형태 다양한 미만성침윤성폐질환에서 수많은 직경 5 mm 이하의 작은 결절들이 산재하여 보인다(Fig. 8). 결절음영 형태를 보 이는 감염성 질환들로는 속립성 결핵, 히스토플라스마증 (histoplasmosis), 콕시디오이데스진균증(coccidioidomycosis), 패혈증성 색전증(septic emboli)등이 있고, 갑상선암 또 는 췌장암의 폐전이, 세기관지폐포암, 유육종증(sarcoidosis), 규폐증(silicosis), 탄광부 진폐증 등에서 결절음영 형태를 보 인다. Fig. 9. Silicosis in a 66-year-old man. Chest radiograph shows characteristic nodular pattern with upper lung zone predominance. The nodules are typically very sharply marginated and progressive massive fibrosis is seen in upper lung zone with peripheral cicatricial emphysema. These findings are very important clues to this diagnosis. He was a mine worker over 20 years. Fig. 7. Langerhans cell histiocytosis in a 33-year-old man. Moderate-sized cystic spaces are seen in mid and upper lung zone in a central distribution on chest radiograph. The history of heavy smoking suggests Langerhans cell histiocytosis to the top of the differential diagnosis and the diagnosis was proven by surgical lung biopsy. Fig. 8. Nodular pattern (left) vs. reticular pattern (right) on chest radiographs. White nodules are round and intervening black spaces are irregular in nodular pattern. Black holes are round and the surrounding interstitial fibrosis is irregular in reticular pattern. Fig. 10. Lung metastasis in a 68-year-old woman. Chest radiograph demonstrates numerous small nodular densities diffusely scattered in both lungs predominantly distributed in lower lung zone. 506
5 Fig. 11. Pneumocystis carinii pneumonia in a 53-year-old man with AIDS. Chest radiograph shows slightly increased overall opacity of the lungs. Pulmonary vascular markings are indistinct due to ground-glass opacities. Pneumocystis carinii pneumonia is the most common cause of ground-glass pattern in patients with AIDS. 507 Fig. 12. Pulmonary alveolar proteinosis in a 66-year-old man. Chest radiograph demonstrates diffuse ground-glass opacities in both lungs with a radiologic feature similar to pulmonary edema. However, absence of cardiomegaly or pleural effusions and insidious onset of mild symptoms are helpful to the diagnosis. The diagnosis was confirmed with bronchoalveolar lavage.
6 강은영 외: 미만성침윤성폐질환 진단의 영상의학적 접근 분포를 파악하는 것이 중요하다. 고해상CT에서 미만성침윤성 폐질환의 형태는 폐소엽간중격 비후, 망상음영, 낭성음영, 결 절음영, 간유리음영, 폐경화 형태로 구분하였다(5, 7). 폐소엽간중격 비후 형태 폐소엽간중격 비후는 주변부 폐야에서는 흉막에 수직인 선 음영으로 보이며 중심부 폐야에서는 다각형 모양(polygonal arcades)으로 보인다. 폐소엽간중격 비후는 폐간질에 수분 또 는 세포침착, 섬유화, 때로는 중격 주변부의 병변에 의한다. 고 해상CT에서 평탄한(smooth), 결절양(nodular), 또는 불규칙 한(irregular)형태로 나타난다(Fig. 13) (8). 다양한 많은 질환에서 폐소엽간중격 비후를 동반한다. 그러 나 폐소엽간중격 비후가 주 소견인 질환으로는 정수압성 폐부 종과 림프관성폐전이암이 있다. 간질성폐부종은 특징적으로 평 탄한 폐소엽간중격 비후를 일으킨다. 림프관성 폐전이암은 평 탄한 또는 결절양 비후를 보이고, 이는 폐간질내 림프관의 부 종과 종양의 증식을 반영한다(Fig. 14). 불규칙한 비후는 폐 섬유화를 동반한 비후를 반영하며 폐실질의 왜곡을 동시에 볼 수 있다. 된 간유리음영 형태를 동반하기도 한다(Fig. 15). 고해상CT에 서 특발성폐섬유화증과 동일한 형태와 분포를 보이는 질환으 로 결체조직질환과 동반된 폐섬유화증이 있고, 특히 진행성전 신성경화증과 류마티스성관절염 환자에서 흔히 보인다. 만성 과민성폐렴에서 망상음영 형태는 늑횡격막각 부분은 비교적 침범하지 않고 폐소엽중심성(centrilobular) 결절들이 있으므 로 특발성폐섬유화증과 감별된다. 석면폐증도 망상음영 형태 를 보이며, 흉막하 선음영, 폐실질 밴드(parenchymal band), 벌집양폐 등의 소견을 동반한다. 폐 기저부와 흉막하 분포는 망상음영 형태 고해상CT에서 망상음영 형태는 폐 실질내의 불규칙한 선들 과 혈관, 기관지, 장측흉막과 폐실질 사이의 비정상적인 접촉 면에 의해 생긴다. 이러한 형태는 가장 흔히 폐섬유화를 동반 하는 미만성침윤성폐질환에서 볼 수 있다. 폐섬유화에 의한 다 른 소견들로는 폐실질의 왜곡과 기관지 또는 세기관지의 확장 이 있다. 망상음영 형태를 보이는 만성 미만성침윤성폐질환으 로는 특발성폐섬유화증, 결체조직질환과 동반한 폐섬유화증, 석면폐증, 만성 과민성폐렴이 있다. 특발성폐섬유화증은 특징적으로 망상음영 형태가 하부 폐야 그리고 흉막하 주변부 폐야에 분포한다. 벌집양 폐 또는 국한 Fig. 14. Lymphangitic carcinomatosis in a 50-year-old woman with adenocarcinoma of the lung. High-resolution CT shows smooth and nodular interlobular septal thickening as well as bronchovascular bundle thickening. Absence of distortion of lung parenchyma and unilateral predominance are helpful to this diagnosis. Although high-resolution CT findings were consistent with lymphangitic carcinomatosis, the diagnosis was confirmed with transbronchial lung biopsy. Fig. 13. CT patterns of interlobular septal thickening. Thickening of interlobular septa is caused by interstitial fluid, cellular infiltration or fibrosis. Smooth septal thickening (left) is a finding of interstitial edema. Nodular septal thickening (middle) is a typical finding of lymphangitic carcinomatosis. Irregular septal thickening (right) with parenchymal distortion is a finding of usual interstitial pneumonia. 508
7 대한영상의학회지 2006;54: 특발성폐섬유화증에 비해 더 뚜렷한 소견이다. 또한, 대부분의 환자에서 흉막 플라크, 미만성 흉막비후를 관찰할 수 있으며, 이러한 흉막비후 소견들과 망상음영 형태의 조합은 석면폐증 의 특징적인 소견이다(Fig. 16). 낭성음영 형태 낭성음영 형태는 2-3 mm에서 1 cm 정도의 직경을 갖는 얇은 벽의, 경계가 분명한, 공기가 찬 낭성 공간으로 이루어진 것을 일컫는다(Fig. 17). 고해상CT에서 낭성음영 형태는 망상 음영 형태와 쉽게 감별되나, 단순흉부X선 사진에서는 음영들 이 겹쳐 보이므로 폐의 작은 낭포 들은 망상음영 형태로 인지 된다. 고해상CT에서 낭성음영 형태로 보이는 질환들로는 특 Fig. 15. Idiopathic pulmonary fibrosis in a 70-year-old man. High-resolution CT through the lower lung zone shows coarse reticular pattern and ground-glass attenuation with predominantly subpleural distribution. The patient showed classic appearances of idiopathic pulmonary fibrosis clinically and radiologically. 발성폐섬유화증, 랑게르한스세포조직구증, 림프관근종증 등이 있다. 특발성폐섬유화증의 낭성음영 형태는 폐섬유화 말기의 심한 벌집양 폐가 낭성음영 형태로 보일 수 있다. 랑게르한스세포 조직구증은 원인을 모르는 드문 질환이다. 조직소견상 초기에 는 랑게르한스 조직구와 호산구가 결절양으로 침윤하며, 말기 에는 섬유화에 의해 세포성분이 대치된다. 젊은 남자에서 호 발하며, 90% 이상에서 흡연력이 있다. 낭성음영 형태로 보이 고 작은 결절들을 동반할 수 있다. 결절들은 특징적으로 질환 의 초기에 나타나며 낭성음영은 말기에 보인다. 또한, 특징적 으로 중상부 폐야를 침범하며, 하부 폐야, 우중엽, 설상엽 (lingular segments)의 침범은 상대적으로 적다. 20% 이상에 Fig. 16. Asbestosis in a 68-year-old man. High-resolution CT shows fine reticular pattern in subpleural and lower lungs. Peripheral reticular pattern, pleural plaque and occupational history indicate a diagnosis of asbestosis. Fig. 17. Differential diagnosis of cystic airspaces. Cysts (middle) are different from emphysema (left) by larger size, smaller number, lack of a centrilobular location, and presence of definable walls. Cysts (middle) are different from honeycomb cyst (right) by larger size, lack of a subpleural distribution, and lack of associated fibrosis. 509
8 강은영 외: 미만성침윤성폐질환 진단의 영상의학적 접근 서 기흉을 일으키고, 폐용적은 정상이거나 오히려 증가한다. 임 상적 배경이 맞으면 고해상CT소견으로 확진이 가능하다(Fig. 18). 림프관근종증은 기관지, 세기관지, 폐포벽, 폐동맥, 림프 관, 흉막에 있는 평활근이 증식하는 아주 드문 질환이다. 특징 적으로 가임기의 여성에서 발생한다. 고해상CT에서 정상 폐 실질 사이에 얇은 벽의 2 mm-5 cm 크기의 낭포들이 산재해 있으며, 전 폐야에 고르게 분포한다. 폐 용적은 낭성 공간들과 공기포획으로 점차 증가한다. 환자의 1/2-1/3에서 기흉이나 유미흉을 동반한다. 결절성경화증 환자의 극히 일부에서 림프 관근종증과 동일한 폐질환을 일으키기도 한다. 림프관근종증 Fig. 18. Langerhans cell histiocytosis in a 21-year-old woman. High-resolution CT at the level of carina shows numerous cysts and small nodules in both lungs. Cysts are thin-walled and some cysts are bizarre in shape. Irregular and bizarre shaped cysts, small centrilobular nodules, and predominantly upper lung involvement in a heavy smoker permit a confident diagnosis of Langerhans cell histiocytosis which was confirmed with surgical lung biopsy. Fig. 19. Lymphangioleiomyomatosis in a 43-year-old woman. High-resolution CT shows numerous thin-walled cysts randomly distributed throughout both lungs. Cysts are round and the wall is barely perceptible. Incidentally chest drainage tube is noted in right hemithorax for pneumothorax. Thin-walled cysts with uniform distribution and pneumothorax in a woman of childbearing age are pathognomonic for lymphangioleiomyomatosis. 은 결절이 보이지 않고, 전 폐야에 고르게 분포하며, 가임기 여 성에서 발생하므로 랑게르한스세포조직구증과 감별할 수 있다 (Fig. 19). 결절음영 형태 1-10 mm 크기의 작은 결절들이 미만성으로 분포하는 경 우 결절음영 형태로 인지한다. 결절음영 형태를 보이는 질환 으로는 유육종증, 폐전이암, 규폐증 및 탄광부 진폐증, 속립성 결핵이 있다. 유육종증은 기관지혈관다발(bronchovascular bundle)내의 림프관, 장측흉막, 폐소엽간중격 등에 비건락화 육아종이 있어, 고해상CT에서 기관지, 혈관, 폐소엽간 중격의 결절양 비후와 결절들로 보이며 림프관 주변부위에 분포한다. 이러한 결절들 Fig. 20. Sarcoidosis in a 48-year-old woman. High-resolution CT shows numerous well-defined interstitial nodules in both lungs. Nodules are distributed along the bronchovascular bundles, interlobar fissure, and subpleural regions reflecting the perilymphatic distribution. The diagnosis was confirmed with transbronchial lung biopsy. Fig. 21. Metastasis from adenocarcinoma of the lung in a 68year-old woman. High-resolution CT shows numerous nodules. Nodules are well-defined and random and diffusely distributed. Lung metastasis was confirmed with transbronchial lung biopsy. 510
9 대한영상의학회지 2006;54: ). 은 크기가 5 mm이하이고, 폐의 중상부에 더 많이 분포한다 (Fig. 20). 림프관성폐전이암의 결절들은 유육종증과 유사하게 림프관 주변부위에 분포하며, 혈행성전이암의 결절은 무작위 로 미만성으로 분포한다(Fig. 21). 규폐증은 이산화 규소를 포 함한 분진을 흡입한 결과로 일어난 폐의 섬유화 질환이다. 고 해상CT에서 특징적으로 작은 결절들이 무작위로 분포하고 폐 의 중상부 특히 후반부 폐야에 산재한다. 심한 경우에는 이러 한 결절들의 크기와 숫자가 증가하고, 결절들의 융합이 일어 난다. 속립성결핵은 1-3 mm 크기의 경계가 뚜렷한 또는 불 분명한 결절들이 전 폐야에 산재하고, 기관지와의 연관성은 없 다. 폐소엽내 또는 폐소엽간중격 비후를 동반할 수 있다(Fig. 간유리음영 형태는 혈관음영을 상쇄하지 않은 증가한 음영 을 말한다. 비 특이적인 소견이며, 폐포의 부분적인 충전, 폐 간질의 비후, 폐포의 부분적 허탈, 호기상태, 모세혈관내 혈류 의 증가에 의한다. 간유리 음영 유무를 판단하기 어려울 때는 폐실질의 농도와 기관지내의 공기를 비교하면 판단에 도움이 되기도 한다. 간유리 음영을 보이는 미만성침윤성폐질환으로 는 비특이성간질성폐렴(nonspecific interstitial pneumonia), 호흡성 세기관지염-간질성폐질환, 박리성간질성폐렴, 림프구 Fig. 22. Miliary tuberculosis in a 33-year-old man. High-resolution CT shows randomly distributed innumerable tiny nodules throughout both lungs, expression of hematogenous dissemination of the disease. Transbronchial lung biopsy was performed from the lateral basal segment of right lower lobe and showed chronic caseous granulomatous inflammation. Fig. 24. Summer-type hypersensitivity pneumonitis in a 48year-old woman. High-resolution CT shows ground-glass attenuation and airtrapping in both lungs. The combination of a mosaic pattern of ground-glass attenuation and centrilobular nodules is suggestive of the diagnosis. The diagnosis was confirmed with surgical lung biopsy. Fig. 23. Nonspecific interstitial pneumonia in a 61-year-old woman. High-resolution CT shows ground-glass attenuation in both lungs. The abnormalities involve mainly mid to lower lung zones with a subpleural distribution. The diagnosis was confirmed with surgical lung biopsy. Fig. 25. Pneumocystis carinii pneumonia in a 44-year-old man with AIDS. High-resolution CT shows diffuse ground-glass attenuation with some smooth interlobular septal thickening in both lungs. The diagnosis was confirmed with cytopathology of bronchial washing fluid. 간유리음영 형태 511
10 Fig. 26. Diffuse pulmonary hemorrhage associated with propylthiouracil (PTU) induced antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis in a 57-year-old woman. High-resolution CT shows diffuse ground-glass attenuation with smooth inter- and intra-lobular septal thickening. This pattern is so-called crazy-paving pattern. Bronchoscopy and bronchoalveolar lavage revealed pulmonary hemorrhage. Her hemoglobin level dropped to 4.7 g/dl. Fig. 27. Cryptogenic organizing pneumonia in a 53-year-old woman. High-resolution CT shows bilateral patchy air-space consolidation with a predominantly peribronchovascular distribution. Surgical lung biopsy specimen obtained from right lower lobe showed scattered areas of organizing pneumonia. 1. Muller NL, Miller RR. Computed tomography of chronic infiltra- tive lung disease part I. Am Rev Respir Dis 1990;142: Muller NL, Miller RR. Computed tomography of chronic infiltrative lung disease part II. Am Rev Respir Dis 1990;142: Mathieson JR, Mayo JR, Staples CA, Muller NL. Chronic diffuse infiltrative lung disease: comparison of diagnostic accuracy of CT and chest radiography. Radiology 1989;171: ,,,,,,. : HRCT X. 1996;43: Kang EY, Muller NL. The radiographic and high-resolution computed tomographic findings in interstitial lung disease. In Freundlich IM, Bragg DG. A radiologic approach to diseases of the chest. 2nd ed. Baltimore: Williams & Wilkins 1997: Miller WT Jr. Chest radiographic evaluation of diffuse infiltrative 512
11 lung disease: review of a dying art. Eur J Radiol 2002;44: Screaton NJ, Hiorns MP, Muller NL. Differential diagnosis in chronic diffuse infiltrative lung disease on high-resolution computed tomography. Semin Roentgenol 2002;37: Kang EY, Grenier P, Laurent F, Muller NL. Interlobular septal thickening: patterns at high-resolution computed tomography. J Thorac Imaging 1996;11: Heyneman LE, Ward S, Lynch DA, Remy-Jardin M, Johkoh T, Muller NL. Respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease, and desquamative interstitial pneumonia: different entities or part of the spectrum of the same disease process? AJR Am J Roentgenol 1999;173: Glazer CS, Rose CS, Lynch DA. Clinical and radiologic manifestations of hypersensitivity pneumonitis. J Thorac Imaging 2002;17: Radiologic Approach to Diffuse Infiltrative Lung Disease 1 Eun-Young Kang, M.D., Ok Hee Woo, M.D., Hwan Seok Yong, M.D., Ki Yeol Lee, M.D., Yu-Whan Oh, M.D., In Ho Cha, M.D. 1 Department of Radiology, College of Medicine, Korea University, Korea University Guro Hospital The Radiology approach to diffuse infiltrative lung disease (DILD) is based on a chest radiography and CT. Chest radiography can categorize DILD into five main patterns of abnormality: linear, reticular, cystic, nodular, and ground-glass patterns. The CT patterns of DILD can be classified into six patterns including thickened interlobular septa, reticular, cystic, nodular, ground-glass, and consolidation patterns. The pertinent differential diagnosis of DILD is based on the pattern recognition approach of abnormalities, and a specific diagnosis can often be made using chest radiography and CT. This pictorial essay illustrates the radiology pattern recognition approach for DILD using chest radiography and CT. Index words : Lung, diseases Lung, interstitial disease Lung, radiography Lung, CT Computed tomography (CT), high-resolution Address reprint requests to : Eun-Young Kang, M.D., Department of Radiology, Korea University Guro Hospital, 97 Guro-dong, Guro-ku, Seoul , Korea Tel Fax keyrad@korea.ac.kr 513
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