igg4相關(guān)性疾病的影像改變

1、IgG4相關(guān)性疾病的影像改變,福建醫(yī)科大學附屬第一醫(yī)院 影像科 胡建平,IgG4相關(guān)性疾病的歷史及概念,1961年 Sarles 首次報道有自身免疫特征的胰腺慢性炎癥硬化。1995 年 Yoshida 正式提出AIP概念。2001年 Hamano 指出IgG4水平升高對AIP有重要診斷及鑒別診斷意義。2003年Kamisawa發(fā)現(xiàn)AIP患者胰腺外器官或組織(如膽道、腹膜后、唾液腺等)內(nèi)亦見相同的病理表現(xiàn)，引入了IgG4相關(guān)自身免疫

2、性疾病概念。2010年日本專家達成共識：將此類疾病定義為IgG4相關(guān)性疾病。,IgG4相關(guān)性疾病,累及多器官或組織的慢性進行性自身免疫性疾病。該疾病以血清IgG4升高及IgG4+漿細胞廣泛浸潤多器官或組織為特征，其臨床譜廣泛。血清IgG4細胞水平顯著增高(>1350mg／L),IgG4陽性淋巴細胞在組織中浸潤(IgG4陽性淋巴細胞占淋巴細胞的50％以上)。,IgG4相關(guān)性疾病,特征性病理改變?yōu)榻M織及多個器官中廣泛的IgG4陽

3、性淋巴細胞浸潤，進而導致硬化和纖維化；一個或多個器官或組織腫脹增大，似腫瘤性；對糖皮質(zhì)激素治療反應(yīng)良好。,IgG4相關(guān)性疾病,中老年男性好發(fā)，臨床表現(xiàn)取決受累器官或組織。 胰腺(自身免疫性胰腺炎) 膽道（IgG4相關(guān)性硬化性膽管炎） 腎(間質(zhì)性腎炎) 肺(間質(zhì)性肺炎) 頭頸部(唾液腺和淚腺/垂體/眼眶/腦膜) 其他器官(心血管系統(tǒng)/胃腸道/腹膜后間隙),自身免疫性胰腺炎（autoimm

4、une pancreatitis，AIP),IgG4相關(guān)性疾病在胰腺的局部表現(xiàn)，受累器官見大量淋巴、漿細胞及IgG4陽性細胞浸潤。以胰腺淋巴細胞及漿細胞浸潤并發(fā)生纖維化、影像學表現(xiàn)胰腺腫大和胰管不規(guī)則狹窄、血清IgG4水平升高、類固醇激素療效顯著為特征。,國外報道AIP病例數(shù)約占同期CP的2%-10%，我國報道比例3.6%-9.7%。男女比例約2:1，多見于老年人，大部分患者初次發(fā)病年齡超過50歲，但也可于青少年期發(fā)病。易被誤診為胰

5、腺癌，因疑診胰腺癌而行手術(shù)切除最常見的良性病變，約占所有胰十二指腸切除術(shù)的2.5％左右。,自身免疫性胰腺炎（autoimmune pancreatitis，AIP),自身免疫性胰腺炎（autoimmune pancreatitis，AIP),早期臨床癥狀輕微而無特異性，故早期診斷比較困難?？捎休p微腹痛、周身不適、四肢乏力、惡心、厭食等癥狀，部分患者有阻塞性黃疸(多由于胰腺頭部炎癥腫脹壓迫遠端膽管導致狹窄所致)。,影像學表現(xiàn),形態(tài)學改變

6、 彌漫型：彌漫腫大呈臘腸樣，小葉輪廓消失，邊界清楚，胰周炎癥或纖維化表現(xiàn)為環(huán)形影或“暈征”。局灶型：局灶性腫塊。胰頭常見，邊界清晰，部分見環(huán)形或“暈征”。多灶型；多發(fā)的腫塊樣表現(xiàn)。 CT上表為低密度，MRIT1WI低信號， T2WI信號輕度增高，早期強化不明顯，均勻或不均勻延遲強化；胰周環(huán)形影呈低密度，T1WI及T2WI低信號。胰管彌漫性、不規(guī)則狹窄，狹窄胰管無梗阻或輕度擴張．胰腺段膽總管可見不規(guī)則狹窄。,Different

7、 patterns of autoimmune pancreatitis,Vlachou et al， RadioGraphics 2011; 31:1379–1402,Vlachou et al， RadioGraphics 2011; 31:1379–1402,Diffuse autoimmune pancreatitis in a 75-year-old man,Follow-up CT image obtained 8 mont

8、hs later, after steroid treatment.,F-43，間斷上腹部疼痛，肝酶升高，胰管擴張；實驗室檢查：IG4/IG2升高，脂肪酶升高；CEA,CA199正常,M-75，上腹部疼痛.,focal autoimmune pancreatitis,Kawamoto et al RadioGraphics 2008; 28:157–170,診斷標準,2002年 日本胰腺學會首次提出AIP診斷標準。2006年 日本、

9、韓國、美國 發(fā)布AIP診斷標準。2010年 國際胰腺協(xié)會 AlP診斷標準國際共識。2012年 我國自身免疫性胰腺炎共識意見。,2010年 AlP診斷標準國際共識,診斷依據(jù)包括影像學(細分為胰腺實質(zhì)影像學和胰管影像學)、血清學、胰腺外器官受累、組織病理學和診斷性激素治療等5個方面。特點：強調(diào)胰腺實質(zhì)影像學檢查(CT/MRI)在AlP診斷中的首要地位。實驗室檢查指標僅有IgG4一項。評價診斷性激素治療效果的指標主要依靠影像學，實

10、驗室檢查指標不再作為監(jiān)測指標。,患者有典型影像學征象，且有實驗室檢查或胰腺外受累證據(jù)，即可診斷AIP，可行激素治療。 如影像學不典型，需除外胰腺癌，再結(jié)合實驗室檢查、組織病理學證據(jù)做出診斷。 如行診斷性激素治療，必須除外胰腺癌，療程不長于2周；復(fù)查影像學提示胰腺或胰腺外病變明顯好轉(zhuǎn)者支持AIP診斷。,IgG4相關(guān)性硬化性膽管炎,最常累及的部位為胰腺段膽總管；臨床表現(xiàn)主要為梗阻性黃疸、體質(zhì)量減輕及腹部不適等，并常合并A

11、IP ；與原發(fā)性硬化性膽管炎在臨床及影像表現(xiàn)方面均有重疊，故兩者鑒別困難。,IgG4相關(guān)性硬化性膽管炎,影像學所見 典型表現(xiàn)為長且連續(xù)性的膽道狹窄，狹窄前膽道?？梢姅U張； 膽道壁呈對稱性的環(huán)周增厚．增厚的膽道壁增強掃描可見強化； 膽囊受累時表現(xiàn)為膽囊壁彌漫性增厚，增強延遲強化。,Follow-up MRCP image obtained 2 years later, after steroid therapy。,A

12、61-year-old man with biopsy proven IgG4-related sclerosingcholangitis mimicking cholangiocarcinoma.,diagnosis of IgG4-related disease following cholecystectomy,A 56-year-old man with intrahepatic IgG4-related sclerosing

13、 cholangitis,IgG4相關(guān)性腎病,腎臟是IgG4相關(guān)性疾病另一常見累及的器官，臨床表現(xiàn)主要為蛋白尿、血尿及腎功能異常等。病理特征是間質(zhì)性腎炎、纖維化伴間質(zhì)內(nèi)多發(fā)斑片或彌漫的淋巴漿細胞浸潤。其極少累及腎小球常合并AIP，如無合并AIP，與其他類型間質(zhì)性腎炎鑒別困難。臨床表現(xiàn)隨糖皮質(zhì)激素治療而好轉(zhuǎn)。,IgG4相關(guān)性腎病的影像學表現(xiàn),4種表現(xiàn)類型： 圓形或楔形的腎皮質(zhì)結(jié)節(jié) 腎外周皮質(zhì)病變 腫瘤樣病變

14、腎盂受累腎皮質(zhì)病變常多發(fā)并雙腎受累，單發(fā)病灶少見。單發(fā)腫瘤樣表現(xiàn)與腎腫瘤鑒別困難。腎盂受累表現(xiàn)腎盂壁彌漫性增厚，腔內(nèi)表面光滑。,IgG4相關(guān)性腎病的影像學表現(xiàn),CT平掃病灶常為低密度，部分可無明顯異常表現(xiàn)，增強掃描早期與腎實質(zhì)相比呈低密度，延遲掃描見輕度強化。經(jīng)治療后，部分小病灶會消失．但大部分病灶會形成疤痕殘留于皮質(zhì)。,Vlachou et al， RadioGraphics 2011; 31:1379–1402,M. Kaw

15、ano Clin Exp Nephrol (2011) 15:615–626,Takahashi et al Radiology: Volume 242: Number 3—March 2007,Takahashi et al Radiology: Volume 242: Number 3—March 2007,M. Kawano Clin Exp Nephrol (2011) 15:615–626,Sunchan Kim, Korea

16、n J Urol 2013;54:209-211,M. Kawano Clin Exp Nephrol (2011) 15:615–626,男，58歲,外院檢查發(fā)現(xiàn)右腎占位。2013年1月CT,2013年7月CT,女 43歲；納差、惡心、皮膚鞏膜黃染2個月。,,IgG4相關(guān)間質(zhì)性肺疾病,多數(shù)患者為中老年男性。多數(shù)肺IgG4相關(guān)性肺疾病伴有或繼發(fā)自身免疫性胰腺炎，但也有單獨累及肺部的報道。,IgG4相關(guān)間質(zhì)性肺疾病,IgG4相關(guān)性肺疾

17、病的影像學模式： ①肺泡間質(zhì)型，伴有蜂窩樣變、支氣管擴張和彌漫性磨玻璃影的改變； ②支氣管血管束和小葉間間隔增厚型； ③實性結(jié)節(jié)或團塊樣損害型； ④以多發(fā)的圓形磨玻璃影為特征的圓形磨玻璃影型。,Inoue et al Radiology: Volume 251: Number 1—April 2009；13個case,Schematic of four types of IgG4-related lung dis

18、ease: (a) solid nodular, (b) round-shaped GGO,(c) alveolar interstitial, and (d) bronchovascular,Inoue et al Radiology: Volume 251: Number 1—April 2009,(a, b) Thin-section CT scans of left lung in a 76-year-old woman (ca

19、se 3) with IgG4-related lung disease demonstrate a solid nodule in the left upper lobe (black arrow). This nodule is accompanied by perinodularspiculation. DiffuseGGOis also shown in the left upper lobe separated by a s

20、olid nodule (white arrows).,(a– d) Thin-section CT scans in a 43-year-old man (case 6) with IgG4-related lung disease.Multiple GGOs are shown in both lungs. Each lesion is well defined and round shaped (arrow).,(a, b) T

21、hin-section CT scans in a 59-year-old man (case 7) with IgG4-related lung disease show honeycombing in both lower lobes (black arrows). Bronchiectasis is also observed in both lower lobes (white arrows). Diffuse GGOs are

22、 found in both middle and lower lobes.,(a, b) Thin-section CT scans in a 59-year-old man (case 9) with IgG4-related disease demonstrate thickening of bronchovascular bundles of the right lung (white arrows). Mild thicken

23、ing of the interlobular septa is also noted (black arrows).,(a, b) Thin-section CT scans in a 59-year-old woman (case 11) with IgG4-related lung disease reveal multiple small nodules in both lungs (white arrows). These n

24、odules distribute in the centrilobular areas. Mild interlobular septal thickening is also identified (black arrows).,IgG4相關(guān)性疾病頭頸部改變,IgG4相關(guān)性橋本甲狀腺炎唾液腺和淚腺(米庫利茲病)眼眶(炎性假瘤)垂體(垂體機能減退綜合癥)腦膜(硬腦膜炎),．,IgG4-related disease in a

25、58-year-old woman. (a) Coronal contrast-enhanced CT scan shows diffuse symmetric swelling of the submandibular glands (arrows). Low-attenuation lesions (arrowheads) are incidentally noted in the thyroid gland. (b, c) Axi

26、al unenhanced (b) and contrast-enhanced (c) CT scans demonstrate diffuse low attenuation of the thyroid gland with poor enhancement (arrowheads), findings that are suggestive of IgG4-related disease.,IgG4-related disease

27、 (hypophysitis) in a 56-year-old man. (a) Coronal T2-weighted MR image demonstrates bilateral swelling of the parotid glands with low-signal-intensity infiltration (arrows). (b) Coronal contrast-enhanced fat-suppressed T

28、1-weighted MR image shows the lesions with homogeneous enhancement (arrows). (c) On a sagittal contrast-enhanced T1-weighted MR image, thickening of the pituitary stalk (arrowhead) is incidentally noted.,Mikulicz disease

29、 in a 67-year-old man. Biopsy revealed IgG4-related sclerosing disease.,Dacryoadenitis in a 68-year-old woman. Unilateral dacryoadenitis can be difficult to differentiate from a lacrimal tumor with imaging alone; however

30、, IgG4- related dacryoadenitis was confirmed at surgical resection.,A 42-year-old man with Mikulicz’s disease. Contrast-enhanced coronal (left, centre) and axial (right) CT images show diffuse enlargement of the homogene

31、ously enhancing lacrimal (black arrows), parotid, and submandibular glands, respectively (black asterisks). The same patient had biopsy-proven IgG4-related sclerosing cholangitis.,Biopsy of a left lower eyelid mass (not

32、shown) and elevated serum levels of IgG4 were used to establish the diagnosis. Histopathological diagnosis of IgG4-related disease was made following biopsy of the periorbital masses.,A 37-year-old man with IgG4-related

33、pituitary infundibulo-hypophysitis and hypertrophic pachymeningitis (same patient as in the top image of Fig 11). Contrast-enhanced axial T1-weighted MRI images reveal (top) enlargement of the enhancing pituitary stalk (

34、white arrow) and (bottom) focal dural thickening within the floor of the middle cranial fossa on the left side (white arrow).,其它器官受累,IgG4相關(guān)性疾病累及腹膜后組織多導致腹膜后纖維化；IgG4相關(guān)性疾病累及血管時表現(xiàn)多樣，可包括主動脈炎、主動脈周圍炎、炎性主動脈瘤等；IgG4相關(guān)性疾病與炎癥性腸病之間

35、的相關(guān)性目前尚不明確。有研究發(fā)現(xiàn)，IBD在AlP病人中的發(fā)病率為6％一17％ ，是普通人群發(fā)病率的12～15倍,Sclerosing mesenteritis in a 73-year-old man.,Follow-up CT image, obtained 3 months later after high-dose oral steroid therapy,Paravertebral mass and periaortitis i

36、n a 55-year-old man.Histopathological diagnosis of IgG4-related disease was made following imaging guided biopsy of the paravertebral soft tissue.,A 52-year-old man with coronary artery involvement in IgG4-related disea

37、se,Contrast-enhanced CT scans (early phase) of IgG4-related perivascular lesion in 55-year-old man .) A paravertebral mass (arrow) was also diagnosed; at needle biopsy, it was diagnosed as IgG4-related disease.,(a, b) Co

38、ntrast-enhanced CT scans (early phase) of IgG4-related perivascular lesion in79-year-old-man (case 14) and (c, d) histologic features.,Contrast-enhanced CT scans(early phase) of IgG4-related perivascular lesion in 70-yea

39、r-old man,An 18-year-old man with IgG4-related autoimmune pancreatitis. Contrast-enhanced axial (top) and coronal (bottom) CT images demonstrate diffuse mild colonic wall thickening (white arrows).Colonoscopy confirmed t