其它一些-結(jié)節(jié)病病因不明系統(tǒng)性肉芽腫性疾病

定病因不明的系統(tǒng)性肉芽腫性疾累及全身多系病理為非干酪樣壞死性上皮細(xì)胞肉目前病因不流行病種族 ACaseControlledEtiologicStudyofSarcoidosis_Heterogeneouspopulation(53.5%White,44.5%Black_Gender(64%,female,_Age(46.5tolessthan40yrand54.5togreaterthan40_Women:greaterincidenceofeye,neurological,nodosum(EN),over40_Men:greaterincidenceof_Blacksubjects:increasedskininvolvement(otherthanEN),eye,liver,andbonemarrowinvolvement,extrathoraciclymphnode臨床表現(xiàn)－癥12-全身癥狀15-20-發(fā)熱15-10-

咳嗽30- 30咳痰10-胸痛15-10-10-神經(jīng)2-臨床表現(xiàn)－受累部肺淋皮膚肉芽腫（凍瘡樣狼瘡）與非肉芽腫（結(jié)節(jié)紅斑肝肝功能輕度異常、肝大脾脾大、脾亢骨溶骨、硬化唾液腺腫大關(guān)節(jié)對(duì)稱行關(guān)節(jié)炎、滑膜炎（膝、踝心肺心病、心率失常5神經(jīng)顱底腦膜炎、Ⅶ、Ⅱ、Ⅸ、Ⅹ、Ⅷ臨床表現(xiàn)－受累部腎、鈣化、腎4鼻/淚腺眼干骨骼肌肌痛、無力喉血液系統(tǒng)淋巴低臨床表現(xiàn)－分臨床表現(xiàn)－分臨床表現(xiàn)－分0正常，可有肺外表 1肺門 大，肺部無異常，可伴或不伴氣管 602肺部彌漫病變，肺門 30 154肺纖維化，無肺門 臨床表現(xiàn)－胸肺門：雙側(cè)對(duì)稱性 腫臨床表現(xiàn)肺門縱膈 腫肺門 旁、胸膜下或沿支氣管血管分布小結(jié)節(jié)征 鈣臨床表現(xiàn)－呼吸系咳嗽、呼 、咳痰、胸繼發(fā)真 肺膨脹不全。肺動(dòng)脈高壓（少見肺功能限制、阻塞、彌鎵－67SACE50-BALF淋巴細(xì)胞增多（鈹 Kbeim-Siltzbachtest2-6周、 細(xì)胞因子及受體sIL2-－病TBLB陽性率70- 皮膚（非結(jié)節(jié)紅斑鑒 －上皮樣肉芽性結(jié)核抗酸染色、培真菌六胺銀染色、培麻學(xué)貓抓病立克次氏嗜酸細(xì)腫瘤病淋巴高丙種球蛋白血

炎性疾結(jié)節(jié) 接觸史、鈹皮膚斑貼試驗(yàn)、巴細(xì)胞轉(zhuǎn)化率檢過敏性肺泡炎病史、特異性抗肉芽腫性血管炎病嗜酸性肉芽異物反應(yīng)性肉芽腫病性膽汁性肝硬化AMA、肝外病克隆氏病消化道外病預(yù)2/3自15-20%不可恢吸衰竭并肺心病和右心與急慢性及分期相治AbsoluteIndicationsfor_Activeoculardisease_Progressivepulmonary_Persistent_Centralnervoussystem_Myocardial_Disfiguringcutaneous_Withquestionofliver治Despitethesideeffects,corticosteroidsarethemainstayoftreatmentSarcoidosis.Theyeffectiveinabout85%ofthepatients.NoconsensusregardingdosageTreatmentsOtherThan-Inhaledcorticosteroids-Immunosuppressantagents:azathioprine,cyclophosphamide,-Otheragents:chlorambucil(苯丁酸氮芥chloroquine,-Recenttreatments:pentoxifylline(己酮可可堿),thalidomide(反應(yīng)停infleximab(TNFα拮抗劑-Surgicaltreatments:lungorheart(LateststudiesnotethatSarcoidrecursinthetransplantedorganThissyndromeusuallypresentswitha lationofclassicsymptoms,whichincludesbilateralhilaradenopathy(BHA),EN,polyarticulararthritisandarthralgias,andcan paniedbyhighfevers.Lofgren’shasanexcellentprognosisandcompleteresolutionislikelywithinoneyear.Biopsyisnotneededtomakethediagnosis.上皮細(xì)胞肉芽腫鑒宿主免疫力與肉芽腫形播散→粟粒樣壞死性肉芽腫→壞 泡結(jié)→非壞死性→結(jié)核球→瘢痕愈合結(jié)免疫力增強(qiáng)上皮細(xì)胞肉芽腫鑒 －壞死壞死性上皮細(xì)胞肉芽腫鑒壞死性上皮細(xì)胞肉芽腫→抗酸染色＋→結(jié)核或非結(jié)核分支桿抗酸染色－→真菌（組織胞漿菌、隱球菌、孢子絲菌銀染伴嗜酸或中性粒細(xì)胞的壞死性細(xì)支氣管炎和柵欄樣組織細(xì)胞肉→抗酸染色－→支氣管中心型肉芽腫PAS或銀染伴或不伴壞死的上皮細(xì)胞肉芽腫和柵欄樣組織細(xì)胞肉芽→抗酸染色－→RA肺內(nèi)表PAS或銀染上皮細(xì)胞肉芽腫鑒 －非壞死非壞死性上皮細(xì)胞肉芽腫→→真菌（PAS或銀染→抗酸染色