其它一些-结节病病因不明系统性肉芽肿性疾病

定病因不明的系统性肉芽肿性疾累及全身多系病理为非干酪样坏死性上皮细胞肉目前病因不流行病种族 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临床表现－症12-全身症状15-20-发热15-10-

咳嗽30- 30咳痰10-胸痛15-10-10-神经2-临床表现－受累部肺淋皮肤肉芽肿（冻疮样狼疮）与非肉芽肿（结节红斑肝肝功能轻度异常、肝大脾脾大、脾亢骨溶骨、硬化唾液腺肿大关节对称行关节炎、滑膜炎（膝、踝心肺心病、心率失常5神经颅底脑膜炎、Ⅶ、Ⅱ、Ⅸ、Ⅹ、Ⅷ临床表现－受累部肾、钙化、肾4鼻/泪腺眼干骨骼肌肌痛、无力喉血液系统淋巴低临床表现－分临床表现－分临床表现－分0正常，可有肺外表 1肺门 大，肺部无异常，可伴或不伴气管 602肺部弥漫病变，肺门 30 154肺纤维化，无肺门 临床表现－胸肺门：双侧对称性 肿临床表现肺门纵膈 肿肺门 旁、胸膜下或沿支气管血管分布小结节征 钙临床表现－呼吸系咳嗽、呼 、咳痰、胸继发真 肺膨胀不全。肺动脉高压（少见肺功能限制、阻塞、弥镓－67SACE50-BALF淋巴细胞增多（铍 Kbeim-Siltzbachtest2-6周、 细胞因子及受体sIL2-－病TBLB阳性率70- 皮肤（非结节红斑鉴 －上皮样肉芽性结核抗酸染色、培真菌六胺银染色、培麻学猫抓病立克次氏嗜酸细肿瘤病淋巴高丙种球蛋白血

炎性疾结节 接触史、铍皮肤斑贴试验、巴细胞转化率检过敏性肺泡炎病史、特异性抗肉芽肿性血管炎病嗜酸性肉芽异物反应性肉芽肿病性胆汁性肝硬化AMA、肝外病克隆氏病消化道外病预2/3自15-20%不可恢吸衰竭并肺心病和右心与急慢性及分期相治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(己酮可可碱),thalidomide(反应停infleximab(TNFα拮抗剂-Surgicaltreatments:lungorheart(LateststudiesnotethatSarcoidrecursinthetransplantedorganThissyndromeusuallypresentswitha lationofclassicsymptoms,whichincludesbilateralhilaradenopathy(BHA),EN,polyarticulararthritisandarthralgias,andcan paniedbyhighfevers.Lofgren’shasanexcellentprognosisandcompleteresolutionislikelywithinoneyear.Biopsyisnotneededtomakethediagnosis.上皮细胞肉芽肿鉴宿主免疫力与肉芽肿形播散→粟粒样坏死性肉芽肿→坏 泡结→非坏死性→结核球→瘢痕愈合结免疫力增强上皮细胞肉芽肿鉴 －坏死坏死性上皮细胞肉芽肿鉴坏死性上皮细胞肉芽肿→抗酸染色＋→结核或非结核分支杆抗酸染色－→真菌（组织胞浆菌、隐球菌、孢子丝菌银染伴嗜酸或中性粒细胞的坏死性细支气管炎和栅栏样组织细胞肉→抗酸染色－→支气管中心型肉芽肿PAS或银染伴或不伴坏死的上皮细胞肉芽肿和栅栏样组织细胞肉芽→抗酸染色－→RA肺内表PAS或银染上皮细胞肉芽肿鉴 －非坏死非坏死性上皮细胞肉芽肿→→真菌（PAS或银染→抗酸染色