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1、Pulmonary Sarcoidosis: Typical and Atypical Manifestations at High- Resolution CT with Pathologic Correlation Recent Advances in Sarcoidosis Chest 2019;結節(jié)病CT典型和不典型表現(xiàn)結節(jié)病進展OrganLungPleuraLymph NodesSkinEyeNasal Mucosa鼻粘膜Larynx喉Bone MarrowSpleenLiver%901-575-90252520515-4050-6060-90OrganKidneyCalcium M
2、etabolismNervous SystemBoneJointsHeartEndocrine GlandsParotid GlandGastrointestinal System%Rare1-25525-505Rare10RareSarcoidosis is a multisystem disorder that is characterized by noncaseous epithelioid cell granulomas, which may affect almost any organ. 結節(jié)病是一種以非干酪樣壞死的上皮細胞肉芽腫為特點的多系統(tǒng)疾病,幾乎可以累及一切器官。Sarc
3、oidosis may be asymptomatic or chronic. It commonly improves or clears up spontaneously自愈或好轉. More than 2/3 of people with lung sarcoidosis have no symptoms after 9 years. About 50% have relapses. About 10% develop serious disability. Sarcoidosis of the lung is primarily an interstitial lung disease
4、 in which the inflammatory process involves the alveoli, small bronchi, and small blood vessels. Typical and Atypical Features of Pulmonary Sarcoidosis at High-Resolution CT Typical features 1 Lymphadenopathy: hilar, mediastinal (right paratracheal), bilateral, symmetric, and well defined 2 Nodules:
5、 micronodules (24 mm in diameter; well defined, bilateral); macronodules (5 mm in diameter, coalescing) 3 Lymphangitic spread: peribronchovascular, subpleural, interlobular septal 4 Fibrotic changes: reticular opacities, architectural distortion, traction bronchiectasis, bronchiolectasis, volume los
6、s Bilateral perihilar opacities 5 Predominant upper- and middle-zone locations of parenchymal abnormalities 結節(jié)病本來是肺間質性病變,累及肺泡,支氣管和小血管淋巴結增大,兩側對稱,境界清楚大小結節(jié)淋巴管播散,支氣管血管鞘,胸膜下,小葉間隔纖維化改動,網(wǎng)狀陰影,肺構造扭曲,牽拉性支擴,肺容積減少,兩肺門旁致密影中上肺為主典型特征 Atypical features 1 Lymphadenopathy: unilateral, isolated, anterior and posterior
7、 mediastinal 2 Airspace consolidation: masslike opacities, conglomerate masses, solitary pulmonary nodules, confluent alveolar opacities (alveolar sarcoid pattern) 3 Ground-glass opacities 4 Linear opacities: interlobular septal thickening, intralobular linear opacities 5 Fibrocystic changes: cysts,
8、 bullae, blebs, emphysema, honeycomb-like opacities with upper- and middle-zone predominance 單側孤立前后縱隔淋巴結增大肺泡實變:腫塊,本質性結節(jié),實變交融毛玻璃陰影線狀陰影小葉間隔增厚小葉年線狀陰影不典型表現(xiàn)囊性纖維化改動:囊,大泡,小泡,肺氣腫,蜂窩樣改動 6 Miliary opacities 粟粒樣改動 7 Airway involvement: mosaic attenuation pattern, tracheobronchial abnormalities, atelectasis 氣道累
9、及:馬賽克改動,氣管支氣管異常,肺不張 8 Pleural disease: effusion, chylothorax, hemothorax, pneumothorax, pleural thickening, calcification Pleural plaquelike opacities 9 Mycetoma, aspergilloma 霉菌球During the past decade, advances have been made in the study of sarcoidosis The Mycobacterium tuberculosis catalase-perox
10、idase (mKatG) protein, a potential antigen, has been identified,結核分枝桿菌過氧化氫酶過氧化物酶mkatg蛋白,作為一個潛在的抗原已被確定。 PET scanning has proven valuable in locating occult sites for diagnostic biopsy. PET掃描已被證明在定位活檢診斷隱匿性病灶的價值 Endobronchial ultrasound- guided transbronchial needle aspiration (EBUS-TBNA) of mediastina
11、l lymph nodes has facilitated diagnosis, often eliminating the need for more-invasive procedures, such as mediastinoscopy. 支氣管內超聲引導下經(jīng)支氣管針吸活檢EBUS-TBNA的縱隔淋巴結有利于診斷,往往防止更具侵襲性的操作,如縱隔鏡。Gene expression analyses have more clearly defined cytokine dysregulation in sarcoidosis Currently, no reliable prognosti
12、c biomarkers have been identified. 目前,還沒有可靠的預后標志物已被確定。The tumor necrosis factor (TNF) inhibitors, a relatively new class of steroid-sparing agents, have been used in patients with refractory disease. It is unclear whether medications used to treat pulmonary arterial hypertension are safe and effecti
13、ve for the treatment of sarcoidosis-associated pulmonary hypertension. 腫瘤壞死因子TNF抑制劑,一個相對較新的類固醇保代理類藥品,已被用于治療難治性疾病。目前還不清楚能否用于治療肺動脈高壓的藥物治療結節(jié)病相關性肺動脈高壓是平安和有效的。Pathologic Correlation 相關病理相關病理 Granulomas in the lung parenchyma have a characteristic distribution in relation to lymphatics in the peribronchov
14、ascular interstitial space, subpleural interstitial space, and, to a lesser extent, the interlobular septa (ie, a lymphangitic distribution) 肺本質肉芽腫分布與支氣管血管鞘,胸膜下結締組織,小葉間隔中淋巴管相關肺本質肉芽腫分布與支氣管血管鞘,胸膜下結締組織,小葉間隔中淋巴管相關. Thickened bronchovascular bundles and small perivascular nodules seen at CT corresponded
15、to granulomas within the connective tissue sheath surrounding pulmonary airways and vessels. Pleural or subpleural nodules were correlated with granulomas adjacent to the visceral pleuraCT上支氣管血管鞘增厚和小結節(jié)是與包繞氣道血管結締組織鞘中肉芽腫,胸膜和胸膜下上支氣管血管鞘增厚和小結節(jié)是與包繞氣道血管結締組織鞘中肉芽腫,胸膜和胸膜下結節(jié)與結節(jié)與臟層胸膜旁肉芽腫相關。臟層胸膜旁肉芽腫相關。 Ground-gl
16、ass opacities represented an accumulation of many granulomatous lesions, with or without fibrosis, in the alveolar septa and around the small vessels. No alveolitis was seen 肺泡間隔小血管周圍大量肉芽腫是毛玻璃陰影主要緣由,可伴纖維化,但沒有肺泡炎。肺泡間隔小血管周圍大量肉芽腫是毛玻璃陰影主要緣由,可伴纖維化,但沒有肺泡炎。 Large parenchymal nodules (1 cm in diameter) repr
17、esented coalescent granulomas 大結節(jié)是肉芽腫病變的交融大結節(jié)是肉芽腫病變的交融 Air bronchiolograms within regions of dense consolidation on CT images corresponded to bronchiolar dilatation with surrounding fibrosis 支氣管充氣癥是纖維化旁的支擴支氣管充氣癥是纖維化旁的支擴 honeycomb-like pattern of microscopic cysts seen at pathologic analysis. 蜂窩樣改動在顯
18、微鏡下就是很多的小囊蜂窩樣改動在顯微鏡下就是很多的小囊 pulmonary sarcoidosis shows the typical perilymphatic distribution of micronodules (arrow). 外周淋巴分布的微結節(jié) Photomicrograph of a lung biopsy specimen demonstrates numerous epithelioid granulomas (arrow) surrounding the bronchial walls and immediately beneath the normal bronchi
19、al epithelium (arrowheads). CT scan shows multiple micronodules with a peribronchovascular distribution in both lungs, predominantly in the upper and middle lobes. One cluster of nodules in the periphery of the left upper lobe (arrow) has coalesced to form a conglomerate lesion (macronodule). Corona
20、l reformatted image from high-resolution CT clearly shows upper-lobe predominance of the micronodules. Low-magnification photomicrograph slice from the lower part of the right upper lobe shows multiple confluent granulomas infiltrating the peribronchovascular (arrows) and subpleural (arrowheads) int
21、erstitium. CT scan shows mediastinal lymph node enlargement and a reticular pattern produced by nodularity and thickening of interlobular septa, pleural surfaces, and fissures, CT掃描顯示縱隔淋巴結腫大和構成網(wǎng)狀圖案的小葉間隔增厚,及胸膜外表,及產生的裂痕。 (b) Photomicrograph of a specimen from fine-needle aspiration biopsy of an enlarg
22、ed right paratracheal lymph node shows a group of histiocytes against a lymphocytic background, a cytologic structure characteristic of sarcoid granuloma. (c) Photomicrograph of a lung biopsy specimen from another patient shows progressive thickening of the interlobular septum (*) because of the acc
23、umulation of numerous sarcoid granulomas (arrowheads), an appearance that correlates well with the CT features seen in a. 從擴展的右氣管旁淋巴結細針穿刺活檢標本顯微鏡下顯示一組以淋巴細胞的背景的組織細胞,具有結節(jié)病肉芽腫細胞的構造特征。C從另一個病人的肺活檢標本的顯微照片顯示小葉間隔增厚*,大量積累的肉芽腫結節(jié)箭頭。 expiratory CT scan obtained in a patient with pulmonary sarcoidosis shows a mos
24、aic pattern consisting of multiple areas of low attenuation (arrows) interspersed with larger areas of normal lung parenchyma.This appearance is produced by air trapping. Photomicrographs of a transbronchial lung biopsy specimen show accumulations of sarcoid granulomas (*) in the mucosal and submuco
25、sal layers of bronchiolar epithelium (arrows in b).呼氣相CT掃描在肺結節(jié)病患者顯示馬賽克征,由低密度的多個區(qū)域箭頭交叉著大面積的正常肺本質。這種景象是由空氣滯留產生。一經(jīng)支氣管鏡肺活檢標本顯示的積累結節(jié)病肉芽腫的顯微照片在粘膜和粘膜下的細支氣管上皮層箭頭B。 . (11a) CT scan shows a diffuse ground-glass pattern produced by multiple confluent micronodules, with associated bronchiectasis. CT掃描顯示彌漫性磨玻璃影,
26、由多個交融的結節(jié)產生,伴支氣管擴張 (11b) Magnified axial high-resolution CT scan of the right lung clearly depicts separate nodules in a subpleural (black arrow) and fissural (white arrow) distribution and along the bronchovascular bundles (arrowheads) 放大的軸位高分辨率CT掃描清楚顯示右肺胸膜下區(qū)分胸膜下的結節(jié)黑色箭頭和沿支氣管血管束分布的“裂白色箭頭. (11c) High-
27、power photomicrograph shows an accumulation of interstitial granulomas (white *), which causes a thickened appearance of the interalveolar septa, and acinar granulomas (black *), which form in the interstitium of the alveolar wall and protrude into the alveoli (arrowheads). 高倍鏡下涂片顯示間質肉芽腫集聚白*,使肺小泡壁的增
28、厚,和腺泡肉芽腫黑色,構成在肺泡壁的間質和伸入肺泡箭頭。Sarcoidosis in a patient with a history of stage III primary cutaneous malignant melanoma. 結節(jié)病III期原發(fā)性皮膚惡性黑色素瘤病史。 (a) Contrast- enhanced CT scans show pulmonary nodules (arrow) in subpleural (right) and fissural (left) regions. A video-assisted thoracoscopic surgical biops
29、y was performed. (b) Low-power photomicrograph (original magnification, 10; H-E stain) obtained at histopathologic analysis shows a subpleural nodule that is darker in color because of anthracosis塵肺. (c) Photo- micrograph obtained at higher power (original magnification, 100; H-E stain) shows multip
30、le nonnecrotic granulomas壞死性肉芽腫 (arrows) expanding the interstitium that surrounds the subpleural nodule in b. Typical (a, b) and atypical (c, d) radiologic findings of lymphadenopathy in four patients with sarcoidosis四例結節(jié)病淋巴結病變的影像學表現(xiàn). (a) Axial contrast materialenhanced CT scan (mediastinal window)
31、 shows typical bilateral and symmetric hilar (ar- rows) and subcarinal (*) lymphadenopathy.CT加強掃描縱隔窗顯示了典型的雙側對稱性肺門箭頭及隆突下淋巴結腫大* (b) Axial unenhanced CT scan (mediastinal window) obtained at the level of the left pulmonary artery shows enlargement of right paratracheal and left hilar lymph nodes (arrow
32、s) 右氣管旁和左肺門淋巴結腫大. Although the right hilum is not shown, it too was affected. (c) Axial unenhanced CT scan (mediastinal window) shows punctate cal- cifications of hilar lymph nodes (arrows), a pattern that also occurs in other chronic granulomatous diseases. (d) Axial contrast-enhanced CT scan shows
33、 bilateral eggshell-like calcifications of hilar and mediastinal lymph nodes雙側縱隔、肺門淋巴結蛋殼樣鈣化 (arrows), findings that warrant the inclusion of silicosis 矽肺in the differential diagnosis in this case. Axial high-resolution CT scans obtained at the level of the upper lobes (a) and carina (b) in a patient
34、 with pulmonary sarcoidosis show a fibrotic-cicatricial pattern of disease, with multiple lesions in a peribronchovascular distribution. Characteristic features of chronic disease are depicted, including traction bronchiectasis牽拉性支氣管擴張, severe architectural distortion, volume loss, and interlobular
35、septal thickening. Coales- cent irregular masslike opacities (white arrows) and a calcified right lower paratra- cheal node (black arrow in b) also are seen. Mosaic attenuation, which is most visible in a, presumably results from airway distor- tion due to fibrosis. Axial high-resolution CT scan sho
36、ws several large, ill-defined nodules and areas of con- solidation resulting from the confluence of multiple parenchymal micronodules composed of numerous tiny granulomas in both lungs. Fine nodular opacities are seen around the large nodules (white arrows), and small low-attenuation spots that corr
37、espond to the spaces between partially coalescent small nod- ules are visible peripherally.This appearance has been termed the sarcoid “galaxy sign小結節(jié)環(huán)繞大結節(jié)銀河征. Distortion of the right major fissure is also seen 扭曲的葉間胸膜(black arrow). Alveolar sarcoid多房型肉瘤 pattern of airspace consolidation in pulmonar
38、y sarcoidosis. Axial high-resolution CT scan shows alveolar consolidation in the left upper lobe and patchy subpleural alveolar opacities in the right upper lobe. Architectural distortion and traction bronchiectasis, signs of fibrosis, also are visible, mainly in the right upper lobe. Axial unenhanc
39、ed high-resolution CT scan shows asymmetric subpleural honeycomb-like cysts不對稱胸膜下蜂窩樣囊腫 (ar- rowheads) and architectural distortion associated with left fissure nodularity (arrow). Although these features also are characteristic of idiopathic pulmonary fibrosis特發(fā)性肺纖維化, the upper-lobe predominance of
40、honeycomb-like cysts and the peribronchovascular and fissural distribution of micronodules in this case were more suggestive of sarcoidosis.以上葉為主的蜂窩狀囊 ,沿支氣管血管束周圍分布并且更提示結節(jié)病。Miliary opacities in sarcoidosis. 粟粒樣結節(jié)病 Axial un- enhanced high-resolution CT scan shows countless tiny micronodules representi
41、ng multiple and diffuse granulomas in a random distribution, with bronchial wall thickening. When this pattern is seen, the differential diagnosis should include miliary tuberculosis, pneumo- coniosis, and metastatic lesions. 無數(shù)的小結節(jié)表現(xiàn)為隨機分布的多發(fā)彌漫性肉芽腫,支氣管壁增厚。鑒別診斷應包括粟粒性肺結核,塵肺,和轉移性病變。A, High-resolution C
42、T scan of lungs reveals ill-defined small nodular opacities in peribronchovascular and issural distribution. Small amount of interlobular septal thickening少量的小葉間隔增厚少量的小葉間隔增厚 (arrow) is seen. B, Photomicrograph of histopathologic specimen shows noncaseating ?tightly formed granulomas and epithelioid histiocytes and lymphocytes. Perilymphatic distribution of
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