纖維素性縱隔炎呼吸與危重癥醫學科陳文慧5/9/20241纖維素性縱隔炎病史、查體女性，74歲，漢族反復咳嗽、咳痰，喘息10余年，加重7天一直診斷為慢性阻塞性肺疾病近一年來活動耐力下降否認結核病史，吸煙史20年，20支/天查體：口唇輕度紫紺，球結膜無水腫，頸靜脈充盈。桶狀胸，雙肺呼吸音減低，呼氣相延長，雙下肺可聞及呼氣相干性啰音，5/9/20242纖維素性縱隔炎輔助檢查血氣分析（未吸氧）：pH7.517,PaCO229.3mmHg，PaO252.2mmHg。血氣分析（吸氧2L/min）：pH7.493,PaCO232.4mmHg，PaO260.8mmHg。阻塞性通氣功能障礙（FEV1/FVC：71.34%，FEV1實/預：85.7%）心臟彩超：肺動脈高壓（重度），TI法估測肺動脈收縮壓105mmHg，5/9/20243纖維素性縱隔炎影像學5/9/20244纖維素性縱隔炎概念縱膈中無細胞成分的膠原和纖維組織的良性增生上腔靜脈、肺動脈、肺靜脈、氣管、食管分類局限：肺門、氣管旁及心包旁；局限、鈣化腫塊；與組織胞漿菌或結核感染有關廣泛：非鈣化腫塊；廣泛區域受侵；可能與腹膜后纖維化有關5/9/20245纖維素性縱隔炎病因硬化性縱隔炎/縱膈纖維化縱膈非特異性纖維炎癥性病變多數與組織胞漿菌感染引起的異常免疫反應有關；遺傳易感性多發生于美國組織胞漿菌的流行區域組織胞漿菌抗原的皮膚反應陽性肉芽腫性炎部分病例組織標本中可找到組織胞漿菌5/9/20246纖維素性縱隔炎纖維素性縱隔炎與縱膈肉芽腫縱膈肉芽腫增大、粘連的淋巴結含干酪或纖維性物質有完整包膜，不引起周圍組織的入侵無癥狀或壓迫上腔靜脈或食管引起的癥狀肉芽腫的破裂引起局部或廣泛縱隔纖維化34%肉芽腫進展為纖維素性縱隔炎5/9/20247纖維素性縱隔炎縱膈肉芽腫Figure1.

Mediastinalgranulomaduetohistoplasmosisina30-year-oldmanwithchestpain.(a)Posteroanteriorchestradiographshowsacalcifiedrightparatrachealmass(arrowhead).(b)CTscan(mediastinalwindow)showsthefocalparatrachealmasswithalow-attenuationcenterandextensivecalcification(arrowhead).Notethemasseffectonthetrachea(T).Anoninvasive,well-encapsulatedmasscontainingviableHcapsulatumorganismswasfoundatresection.5/9/20248纖維素性縱隔炎相關疾病結核、曲霉菌、毛霉菌、芽生菌、隱球菌自身免疫性疾病白塞病風濕熱放療外傷何杰金淋巴瘤非特異性炎癥性病變腹膜后纖維化硬化性膽管炎眼眶假瘤5/9/20249纖維素性縱隔炎病例1-影像學（58歲男性，咳嗽6個月）(a)Computedtomographic(CT)scan(lungwindow)showsaninfiltrating,soft-tissuerighthilarmassextendingintotherightlowerlobealongbronchovascularbundles.(b)CTscan(mediastinalwindow)showsthesoft-tissuemass(arrowhead)andextensivecalcificationintherighthilumandsubcarinalregion.5/9/202410纖維素性縱隔炎病例1-大體病理(c)Photographofthecutsurfaceoftheresectedspecimenshowsdensewhitefibroustissueintherighthilumextendingintothelungparenchymaalongbronchovascularbundles(arrows).5/9/202411纖維素性縱隔炎病理學表現Fibrosingmediastinitis.Medium-powerphotomicrograph(originalmagnification,×25;hematoxylin-eosinstain)demonstratesfibroustissue(arrows)infiltratingmediastinaladiposetissue.

Fibrosingmediastinitis.High-powerphotomicrograph(originalmagnification,×100;hematoxylin-eosinstain)demonstratespaucicellular,eosinophilicmaturecollagen,findingstypicaloffibrosing

5/9/202412纖維素性縱隔炎鑒別診斷局限性纖維化-組織胞漿菌病、結核惡性腫瘤何杰金淋巴瘤非何杰金氏淋巴瘤胸膜纖維性腫瘤—CD34、bcl-2胸膜間皮瘤-keratin轉移癌的纖維炎癥反應胸腺瘤胸腺類癌纖維瘤病肉瘤5/9/202413纖維素性縱隔炎組織胞漿菌病(a)Medium-powerphotomicrograph(originalmagnification,×50;hematoxylin-eosinstain)ofahilarlymphnodespecimenshowsacaseatinggranulomacomposedofarimofepithelioidhistocytes(arrowheads)surroundingcentralcaseousnecrosis(N).Noteperipherallymphoidinfiltrate(L).(b)Oilimmersionphotomicrograph(originalmagnification,×500;Grocottmethenamine-silverstain)showssmalloval-shapedyeastsofHcapsulatum.Notetherarebuddingforms(arrowhead).5/9/202414纖維素性縱隔炎非何杰金氏淋巴瘤(a)High-powerphotomicrograph(originalmagnification,×400;hematoxylin-eosinstain)demonstrateshypercellularatypicallymphoidinfiltrate.(b)High-powerphotomicrograph(originalmagnification,×400)ofaspecimenstainedwithimmunohistochemistryforCD20(B-cellmarker)showspositivecytoplasmicstaining5/9/202415纖維素性縱隔炎臨床表現-15/9/202416纖維素性縱隔炎臨床表現-2上腔靜脈上腔靜脈綜合癥中央氣道咳嗽、呼吸困難反復肺炎肺不張肺靜脈假二尖瓣狹窄癥狀勞力性呼吸困難咯血肺動脈高壓、肺心病肺動脈肺動脈高壓（少見）5/9/202417纖維素性縱隔炎治療和預后死亡原因咯血、肺心病、反復肺部感染治療抗真菌治療激素手術并發癥治療球囊擴張激光治療血管內或氣道內支架5/9/202418纖維素性縱隔炎治療(a)CTscan(mediastinalwindow)showsawiremeshstentintheleftmainbronchus,calcifiedadenopathy(arrow)intheaortopulmonarywindow,andasubcarinalsoft-tissuemass(arrowhead).?=esophagus.(b)CTscan(lungwindow)showsanoutpouching(arrowhead)oftheanterioresophageallumenadjacenttothestentthatwasconfirmedtorepresentabronchoesophagealfistulaatabariumswallowexamination(notshown).Notelingularconsolidation,whichmostlikelyrepresentspneumonia.5/9/202419纖維素性縱隔炎ABCD5/9/202420纖維素性縱隔炎影像學表現5/9/202421纖維素性縱隔炎影像學表現-1(a)33歲女性，胸痛，前縱隔腫物(b)43歲女性，慢性咳嗽、呼吸困難，中、后縱膈腫物5/9/202422纖維素性縱隔炎影像學表現-25/9/202423纖維素性縱隔炎影像學表現(3)-55歲男性，咳嗽、咯血(a)Posteroanteriorchestradiographshowsalefthilarmass.(b)CoronalT1-weighted(imagedemonstratesthelefthilarmass(arrowheads)ofheterogeneouslow-to-intermediatesignalintensityobstructingtheleftmainbronchus5/9/202424纖維素性縱隔炎影像學表現(4)-44歲女性，反復肺炎(a)CTscan(mediastinalwindow)demonstratesacalcifiedrighthilarandmediastinalmassobstructingtherightupperlobebronchus.Notetherightpleuralthickening(arrows),patentbronchusintermedius(arrowhead),andenlargedazygousvein(a).R=rightpulmonaryartery,S=superiorvenacava.(b)AxialT1-weighted(680/20)MRimage

showsaninfiltrativehilarmassofintermediatesignalintensitynarrowingtherightupperlobebronchus(arrow).Notethenarrowedbutpatentsuperiorvenacava(arrowhead)5/9/202425纖維素性縱隔炎影像學表現(5)-22歲男性影像學特征(3)-(a)Contrast-enhancedCTscan(mediastinalwindow)showsasoft-tissueattenuationmassdiffuselyinfiltratingthemediastinum.Noteencasementandnarrowingoftheleftmainbronchus(?)(b)Contrast-enhancedCTscan(mediastinalwindow)obtainedatamorecaudallevelshowsthesubcarinalmass(M),encasementoftheleftmaincoronaryartery(arrow),andnarrowingoftheleftsuperiorpulmonaryvein(S).Not