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IgG4相關(guān)性自身免疫性胰腺炎影像學(xué)表現(xiàn)

2024-04-29 00:44徐兵呂清清陳靜靜
精準(zhǔn)醫(yī)學(xué)雜志 2024年1期
關(guān)鍵詞:X線計(jì)算機(jī)體層攝影術(shù)磁共振成像

徐兵 呂清清 陳靜靜

[摘要]目的分析IgG4相關(guān)性自身免疫性胰腺炎(autoimmune pancreatitis,AIP)的CT和MRI表現(xiàn),以提高對(duì)該病影像學(xué)特征的認(rèn)識(shí),減少影像學(xué)誤診。方法回顧性分析47例IgG4相關(guān)性AIP患者的臨床資料,其中33例行CT平掃及增強(qiáng)掃描,24例行MRI平掃及增強(qiáng)掃描、MRCP檢查,10例聯(lián)合行CT及MRI檢查,觀察病變胰腺的形態(tài)、密度/信號(hào)改變及強(qiáng)化特征、胰腺周圍及胰管改變、胰腺外表現(xiàn)。結(jié)果47例患者中,CT及MRI檢查共觀察到胰腺?gòu)浡阅[大呈“臘腸狀”27例,局灶性腫大20例;CT平掃病變表現(xiàn)為均勻一致的略低密度,增強(qiáng)掃描動(dòng)脈期病變區(qū)呈不均勻斑片狀強(qiáng)化,門脈期和延遲期病變區(qū)呈現(xiàn)較均勻的延遲漸進(jìn)性強(qiáng)化;MRI檢查病變表現(xiàn)為T1WI-FS呈不均勻稍低信號(hào),T2WI呈稍高信號(hào),增強(qiáng)掃描早期病灶不均勻輕度強(qiáng)化,延遲掃描呈較均勻延遲漸進(jìn)性強(qiáng)化;CT及MRI檢查28例胰腺周圍見(jiàn)包膜樣邊緣,即“膠囊征”,增強(qiáng)掃描呈輕中度延遲強(qiáng)化;MRCP檢查示10例患者可見(jiàn)主胰管節(jié)段性狹窄,上游胰管局限性輕度擴(kuò)張;CT及MRI檢查共觀察到30例患者表現(xiàn)為膽管系統(tǒng)改變,可見(jiàn)膽總管胰腺段管壁增厚、狹窄伴延遲強(qiáng)化,MRCP檢查呈“鳥(niǎo)嘴樣”改變,上游肝內(nèi)外膽管不同程度擴(kuò)張。結(jié)論IgG4相關(guān)性AIP的CT和MRI表現(xiàn)具有一定的特征性,結(jié)合影像學(xué)表現(xiàn)及實(shí)驗(yàn)室相關(guān)檢查有助于提高該病的診斷水平。

[關(guān)鍵詞]自身免疫性胰腺炎;免疫球蛋白G4相關(guān)疾?。惑w層攝影術(shù),X線計(jì)算機(jī);磁共振成像;診斷

[中圖分類號(hào)]R576.2;R814.42[文獻(xiàn)標(biāo)志碼]A

Imaging findings of IgG4-related autoimmune pancreatitisXU Bing, LYU Qingqing, CHEN Jingjing(Department of Radiology, The Affiliated Hospital of Qingdao University, Qingdao 266003, China)

[ABSTRACT]ObjectiveTo analyze the computed tomography (CT) and magnetic resonance imaging (MRI) findings of immunoglobulin G4 (IgG4)-related autoimmune pancreatitis (AIP), to improve the understanding of the imaging features of this disease, and to reduce imaging misdiagnosis. MethodsA retrospective analysis was performed on the clinical data of 47 patients with IgG4-related AIP, among whom 33 underwent plain CT scan and contrast-enhanced CT scan, 24 underwent plain MRI scan, contrast-enhanced MRI scan, and magnetic resonance cholangiopancreatography (MRCP) examination, and 10 underwent combined CT and MRI examination to observe the morphology, changes in density/signal, contrast enhancement characteristics of the diseased pancreas, the peripancreatic and pancreatic duct changes, and the manifestations out of the pancreas. ResultsThe CT and MRI scans showed that among the 47 patients, 27 had a “sausage-like”, diffusely enlarged pancreas and 20 had a focally enlarged pancreas. The plain CT scan showed a uniform and slightly low density; the contrast-enhanced CT scan of the lesion showed an inhomogeneous patchy enhancement in arterial phase, and a uniformly delayed progressive enhancement in portal vein phase and delayed phase. The MRI scan of the lesion showed inhomogeneous and slight hypointensity on T1WI-FS and slight hyperintensity on T2WI, the contrast-enhanced MRI scan showed an inhomogeneous mild enhancement in the early stage and a uniformly delayed progressive enhancement on delayed scan. The CT and MRI scans showed that 28 patients had capsule-like edges around the panc-reas, namely “capsule sign”, with a mild to moderate delayed enhancement on contrast-enhanced images. The MRCP showed that 10 patients had segmental stenosis of the main pancreatic duct and localized mild dilatation of the upstream pancreatic duct. The CT and MRI scans showed that 30 patients had changes in the bile duct system, with wall thickening, stenosis, and delayed enrichment of the pancreatic segment of common bile duct. The MRCP showed “beak-like” changes, and dilatation of upstream intrahepatic and extrahepatic bile ducts to varying degrees. ConclusionThe CT and MRI findings of IgG4-related AIP have certain characteristics. The combination of imaging findings and relevant laboratory tests can help improve the diagnosis of this disease.

[KEY WORDS]Autoimmune pancreatitis; Immunoglobulin g4-related disease; Tomography, X-ray computed; Magnetic resonance imaging; Diagnosis

自身免疫性胰腺炎(autoimmune pancreatitis,AIP)是一種特殊的慢性胰腺炎,目前已經(jīng)發(fā)現(xiàn)其有1型和2型AIP兩種類型[1]。1型AIP即IgG4相關(guān)性AIP,最常見(jiàn)于亞洲人群,被認(rèn)為是一種IgG4相關(guān)性疾病在胰腺的表現(xiàn)[2];2型AIP在歐洲和美國(guó)人群中較多見(jiàn),其受累器官的組織病理學(xué)改變與IgG4相關(guān)性疾病不同,而且血清學(xué)檢查不伴有IgG4的異常。IgG4相關(guān)性AIP一般無(wú)特異性臨床癥狀,部分急性發(fā)作患者常常易被誤診為急性胰腺炎[3-4],造成病情反復(fù)發(fā)作,甚至部分患者影像學(xué)表現(xiàn)易被誤診為胰腺癌[5-6],因此準(zhǔn)確診斷具有重要臨床意義。本研究回顧性分析總結(jié)47例經(jīng)病理和(或)臨床證實(shí)的IgG4相關(guān)性AIP的CT及MRI影像學(xué)特點(diǎn),旨在進(jìn)一步提高對(duì)該病的診斷水平。

1資料與方法

收集2013年1月—2018年5月我院收治的47例IgG4相關(guān)性AIP患者的臨床資料,所有納入患者均符合2011年AIP國(guó)際診斷標(biāo)準(zhǔn)[1]。其中33例患者進(jìn)行了CT平掃及增強(qiáng)掃描,24例患者進(jìn)行了MRI平掃及增強(qiáng)掃描、MRCP檢查,10例患者同時(shí)進(jìn)行了CT及MRI檢查。47例IgG4相關(guān)性AIP患者中,男40例,女7例;年齡23~84歲,平均57歲;31例表現(xiàn)為腹痛、腹脹,其中6例同時(shí)伴有黃疸;12例表現(xiàn)為單純黃疸;2例表現(xiàn)為食欲不振、乏力;2例體檢時(shí)發(fā)現(xiàn);4例經(jīng)手術(shù)病理證實(shí);8例經(jīng)ERCP活檢組織和臨床治療證實(shí);39例血清中IgG4水平不同程度升高,采用激素治療有效確診。

對(duì)相關(guān)影像學(xué)資料進(jìn)行回顧性分析,分析內(nèi)容包括胰腺形態(tài)、密度/信號(hào)改變及強(qiáng)化特征、胰腺周圍及胰管改變、胰腺外表現(xiàn)。

2結(jié)果

2.1胰腺形態(tài)學(xué)改變

CT及MRI檢查共觀察到胰腺?gòu)浡阅[大27例,表現(xiàn)為胰腺邊緣飽滿,正常分葉及羽毛狀結(jié)構(gòu)消失,胰尾部短縮,呈“臘腸樣”改變(圖1);局灶性腫大20例,呈“假腫瘤樣”改變(圖2),其中腫大部位位于胰頭部7例,胰頭體部3例,胰體尾部9例,胰尾部1例。

2.2密度/信號(hào)改變及強(qiáng)化特征

CT平掃檢查顯示,病變表現(xiàn)為均勻一致的略低密度,增強(qiáng)掃描動(dòng)脈期病變區(qū)的強(qiáng)化有不同程度減低,呈不均勻斑片狀強(qiáng)化,門脈期和延遲期病變區(qū)呈現(xiàn)較均勻的延遲漸進(jìn)性強(qiáng)化(圖1、2)。MRI檢查表現(xiàn)為T1WI-FS呈不均勻稍低信號(hào), T2WI呈稍高信號(hào),增強(qiáng)掃描早期病灶不均勻輕度強(qiáng)化,延遲掃描呈較均勻延遲漸進(jìn)性強(qiáng)化(圖3)。

2.3胰腺周圍及胰管改變

CT以及MRI檢查28例胰腺周圍可見(jiàn)包膜樣邊緣,即“膠囊征”(圖1、3),其中19例見(jiàn)于彌漫性腫大患者,9例見(jiàn)于局灶性腫大患者,增厚包膜CT平掃呈等或稍低密度,T1WI-FS呈等或稍低信號(hào),T2WI呈低信號(hào),CT及MRI增強(qiáng)掃描呈輕中度延遲強(qiáng)化。MRCP檢查示10例患者主胰管節(jié)段性狹窄,上游胰管可有局限性輕度擴(kuò)張。

2.4胰腺外表現(xiàn)

CT及MRI檢查30例表現(xiàn)為膽管系統(tǒng)改變,其中21例見(jiàn)于彌漫性腫大患者,9例見(jiàn)于局灶性腫大患者,可見(jiàn)膽總管胰腺段管壁增厚、狹窄,MRCP檢查呈“鳥(niǎo)嘴樣”改變(圖3F),增強(qiáng)掃描可見(jiàn)增厚膽總管壁呈延遲強(qiáng)化(圖2),上游膽總管及肝內(nèi)膽管不同程度擴(kuò)張。2例伴有淚腺改變,1例伴有下頜下腺改變,1例同時(shí)表現(xiàn)為腎臟改變,1例伴有脾、胃改變,1例伴有腹膜后纖維化表現(xiàn)。

3討論

1995年,日本學(xué)者YOSHIDA等[7]首先提出了AIP這一概念,認(rèn)為是一種與自身免疫相關(guān)的特殊類型慢性胰腺炎。根據(jù)2011年AIP國(guó)際診斷標(biāo)準(zhǔn)分為2型[1]。1型AIP為淋巴漿細(xì)胞硬化性胰腺炎,表現(xiàn)為顯著的導(dǎo)管周圍淋巴漿細(xì)胞浸潤(rùn)、血清和組織中IgG4陽(yáng)性漿細(xì)胞高水平表達(dá)、席紋狀纖維化和閉塞性靜脈炎,對(duì)激素治療敏感;2型AIP為特發(fā)性導(dǎo)管中心性胰腺炎,其典型特征是胰腺小葉和導(dǎo)管大量的中性粒細(xì)胞浸潤(rùn),被稱為粒細(xì)胞性上皮損害,可導(dǎo)致胰管損傷[8]。依據(jù)患者的病理組織學(xué)以及血清IgG4水平較高且對(duì)激素治療有效的診斷標(biāo)準(zhǔn),本研究所納入的患者均明確為1型AIP,即IgG4相關(guān)性AIP。

IgG4相關(guān)性AIP好發(fā)于老年男性,患者臨床表現(xiàn)多樣,常以黃疸和腹痛起病多見(jiàn),其中無(wú)痛性梗阻性黃疸是最常見(jiàn)的初期癥狀[9],發(fā)熱、厭食、乏力、體質(zhì)量下降均可出現(xiàn),一些患者可伴有其他IgG4相關(guān)胰腺外的組織器官的炎癥(硬化性膽管炎、硬化性涎腺炎、間質(zhì)性肺炎及腹膜后纖維化等)[10-11]。本組47例患者的年齡分布、性別比例與文獻(xiàn)報(bào)道結(jié)果相近,臨床表現(xiàn)則以腹痛為主,其中31例有腹痛、腹脹,18例有黃疸體征,該組中有2例為體檢時(shí)發(fā)現(xiàn),說(shuō)明病變較輕的患者,也可無(wú)明顯臨床癥狀。根據(jù)這些臨床表現(xiàn)并不能明確診斷,血清IgG4對(duì)診斷該病具有一定的特異度和靈敏度[12-13]。本組47例患者中有39例IgG4水平明顯升高,但部分患者的IgG4水平在正常范圍內(nèi)。

在AIP國(guó)際共識(shí)中重點(diǎn)強(qiáng)調(diào)了胰腺實(shí)質(zhì)影像學(xué)檢查在AIP診斷中的主要地位。AIP從形態(tài)上分為彌漫型和局灶型[14]。彌漫型最常見(jiàn),胰腺正常小葉輪廓消失,沿病變胰腺長(zhǎng)軸方向不同程度腫脹、增粗,長(zhǎng)徑顯著大于短徑,呈典型的“臘腸狀”改變,邊界清楚;局灶型表現(xiàn)為局灶性腫塊,以胰頭部常見(jiàn),易與胰腺癌混淆,病灶邊界多清晰。本組47例IgG4相關(guān)性AIP,胰腺病變受累范圍可呈彌漫性腫大,也可呈局灶性腫大,與文獻(xiàn)報(bào)道結(jié)果一致,但局灶性腫大多數(shù)位于胰體尾部,其好發(fā)部位有待于大樣本研究進(jìn)一步證實(shí)。病變區(qū)胰腺的密度均勻,CT平掃時(shí)與正常胰腺的密度相近,MRI檢查T1WI呈稍低信號(hào),T2WI呈稍高信號(hào),這是由于IgG4相關(guān)性AIP很少伴有壞死、囊變、出血、鈣化情況,也不伴脂肪變性或脂肪浸潤(rùn)等病理改變[15]。

本組47例IgG4相關(guān)性AIP,動(dòng)態(tài)增強(qiáng)CT和MRI影像特點(diǎn)呈現(xiàn)一致性,均表現(xiàn)為延遲漸進(jìn)性強(qiáng)化,從平掃到延遲期,病變胰腺的密度、信號(hào)強(qiáng)度逐漸升高,其增強(qiáng)模式與組織病理學(xué)特點(diǎn)有關(guān),病變內(nèi)增生的纖維組織導(dǎo)致微血管受壓、變細(xì),血流速減慢,造影劑通過(guò)時(shí)間延長(zhǎng),因而呈“緩慢漸進(jìn)強(qiáng)化”的特點(diǎn)。漸進(jìn)性強(qiáng)化是IgG4相關(guān)性AIP的一個(gè)特征性表現(xiàn)[16-17]。本研究中大部分胰腺病變周圍可見(jiàn)包膜樣邊緣,即“膠囊征”,此征象在彌漫型AIP中更常見(jiàn),其病理上是一種沿胰腺外圍增生的慢性炎性纖維組織,CT平掃呈等或稍低密度,T1WI呈等或稍低信號(hào),T2WI呈低信號(hào),增強(qiáng)掃描呈輕中度延遲強(qiáng)化,在顯示“包膜樣邊緣”方面,MRI檢查優(yōu)于CT檢查[18-19],“膠囊征”是IgG4相關(guān)性AIP的重要征象,有助于明確診斷[20-21]。另外,由于主胰管的漿細(xì)胞、淋巴細(xì)胞浸潤(rùn)和纖維化,可導(dǎo)致管壁增厚,管腔出現(xiàn)不同程度的狹窄[22]。因此,MRCP顯示IgG4相關(guān)性AIP胰管呈彌漫性不規(guī)則狹窄,很少出現(xiàn)主胰管的顯著擴(kuò)張[23-24],這與胰腺癌不同,本研究也證實(shí)了這點(diǎn)。

膽管是最常受到累及的胰腺外結(jié)構(gòu)[25],被稱為IgG4相關(guān)硬化性膽管炎,多發(fā)生于膽總管胰腺段,其主要的潛在病理改變是淋巴細(xì)胞、漿細(xì)胞浸潤(rùn)及纖維增生引起膽管壁廣泛增厚及纖維化。本組47例IgG4相關(guān)性AIP,30例表現(xiàn)有膽管系統(tǒng)改變,CT及MRI檢查影像學(xué)上表現(xiàn)為膽總管遠(yuǎn)端壁明顯增厚、狹窄,增強(qiáng)掃描呈延遲強(qiáng)化,MRCP檢查表現(xiàn)為逐漸變細(xì),呈“鳥(niǎo)嘴樣”狹窄,近側(cè)膽管有擴(kuò)張[26-27]。而胰腺癌導(dǎo)致的膽總管狹窄呈浸潤(rùn)性不規(guī)則狹窄,遠(yuǎn)端突然中斷[28]。復(fù)習(xí)相關(guān)文獻(xiàn),IgG4相關(guān)性AIP可累及多種臟器[29],如淚腺炎、涎腺炎(兩者同時(shí)存在時(shí)稱Mikulicz?。⒓谞钕傺?、甲狀腺功能減低、間質(zhì)性肺疾病、腹膜后纖維化及腎小管間質(zhì)性腎病等。對(duì)于臨床懷疑IgG4相關(guān)性AIP的患者,試驗(yàn)性激素治療是必要的[30],CT及MRI檢查能直觀對(duì)比病灶的變化,協(xié)助診斷。

總之,IgG4相關(guān)性AIP屬系統(tǒng)性自身免疫性疾病的一部分,屬于慢性增生性炎癥,在CT和MRI影像上均具有特征性改變,結(jié)合實(shí)驗(yàn)室IgG4檢查結(jié)果,可對(duì)大部分病變作出明確診斷,有利于臨床制定有效的治療方案。

作者聲明:徐兵、陳靜靜參與了實(shí)驗(yàn)設(shè)計(jì);徐兵、呂清清、陳靜靜參與了論文的寫作和修改。所有作者均閱讀并同意發(fā)表該論文,且均聲明不存在利益沖突。

[參考文獻(xiàn)]

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(本文編輯 耿波 厲建強(qiáng))

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