复发性多软骨炎

复发性多软骨炎 查看( 537 ) / 评论( 2 ) / 评分( 5 / 0 ) 摘自<中华风湿病学杂志> 复发性多软骨炎,Relapsing Polychondritis～RP,是一种较少见的炎性破坏性疾病～其特点是软骨组织复发性退化性炎症～表现为耳、鼻、喉、气管、眼、关节、心脏瓣膜等器官及血管等结缔组织受累。复发性多软骨炎的病因及发病机制目前仍不清楚～软骨基质受外伤、炎症、过敏等因素的影响暴露出抗原性～导致机体对软骨局部或有共同基质成分的组织,如巩膜、葡萄膜、玻璃体、视神经内膜及神经束膜、主动脉中层和内层的结缔组织、心瓣膜、心肌肌纤维膜、气管黏膜下基底膜、关节滑膜和肾小球及肾小管基底膜等,的免疫反应。复发性多软骨炎可与类风湿关节炎、系统性血管炎、系统性红斑狼疮以及其他结缔组织病并发。各年龄阶段均可发病～好发年龄为30~60岁～发病无性别倾向。病初常为急性炎症～经数周至数月好转～以后呈慢性反复发作。晚期因起支撑作用的软骨组织遭破坏～出现松软耳、鞍鼻以及嗅觉、视觉、听觉和前庭功能障碍。 一、临床表现 复发性多软骨炎可隐匿起病～也可骤发或病情突然加重。活动期可有发热、局部疼痛、疲乏无力、体重减轻和食欲不振等。常见临床表现如下。 1(耳软骨炎:耳廓软骨炎是最常见的临床表现。病变多局限于耳廓软骨部分～包括耳廓、耳屏～有时可侵犯外耳道～常对称性受累～但耳垂不受累。初期仅表现为耳廓红、肿、热、痛、有红斑结节～常在5~10d内自行消退～可反复发作～久之耳廓塌陷畸形～局部色素沉着。耳廓软骨炎可导致耳松软、变形、弹力减弱～出现结节～外耳道萎缩。外耳道狭窄、中耳炎症、咽鼓管阻塞可致传导性耳聋。后期可累及内耳～表现为听觉或前庭功能损伤。病变累及迷路可导致旋转性头晕、眼球震颤、共济失调、恶心及呕吐等。 2(鼻软骨炎:约3/4的患者有鼻软骨炎。在急性期表现为局部红肿、压痛～常突然发病～颇似蜂窝组织炎～数天后可缓解。反复发作可引起鼻软骨局限性塌陷～发展为鞍鼻畸形～甚者在发病1~2d内鼻梁可突然下陷。患者常有鼻塞、流涕、鼻出血、鼻黏膜糜烂及鼻硬结等。 3(眼部病变:眼部受累可单侧或者双侧～表现为突眼、巩膜外层炎、角膜炎或葡萄膜炎。巩膜炎反复发作可导致角膜外周变薄～甚至造成眼球穿孔。此外还可有球结膜水肿、结膜炎、角膜结膜炎、眼干燥、白内障、虹膜睫状体炎、眼外coupling Centre shall be checked, the second poured concrete strength of more than 70%. In the process of reaming or boring, apart from the special provisions in factory, does not allow any work that may affect the coupling Centre, such as irrigation, to cylinder connections to flood the condenser pipes and so on. Well boring, hinge bolt hole flange with coupling of vertical, not oblique. Mounting bolts should be added lubricants, typing gently with a small hammer, not too loose or too tight, packed the bolts with the corresponding seal screw hole should be marked. 6.4.5.4.2 coupling reamed and pairings is close to the diameter of bolt bearing good hinge holes, dressed with a good two coupling bolts before you can move the rotor, boring-reaming hole in turn. Coupling bolts must be tight coupling is symmetrical diameter, in boring, reaming, two bolts out. Well boring, hinge bolt hole flange with coupling of vertical, not oblique. Due to the coupling bolts in accordance with hinge holes corresponding to the diameter of the screw holes one by one after processing, processing back bolt installation location cannot be changed, so according to bolt after the actual weight difference of nut and lock washer to match. General requirements for coupling two symmetrical diameter bolt, nut and lock washer's total weight difference should not be greater than 10 g and knocks stamped mark. (See Figure 18) The coupling bolts apply lubricant, and then gently with copper home, not too loose or too tight. Bolt Assembly is finished, symmetrical tightening all bolts to 30%, 50%, 70%, 100% force, and check the coupling flutter and before connecting the circle gradually changes its value should be less than 0.02mm. Tightening torque of the coupling bolts shall comply with the technical requirements manufacturers drawings, with a dynamometric wrench or log tension tools. Three wheel rotor swing test coupling connections should be checked: taking leave, 直肌麻痹等表现。视网膜病变如视网膜微小动脉瘤、出血、渗出～静脉闭塞、动脉栓塞也常有发生。视网膜血管炎或视神经炎可导致失明。随着病情的反复发作～患者常可同时有几种眼疾。 4(关节损害:复发性多软骨炎的关节损害特点是外周关节非侵蚀性非畸形性多关节炎。大小关节均可受累～呈非对称性分布～多为间歇性发作～慢性持续性者较少。肋软骨和胸锁关节以及骶髂关节也可受累。此外尚可发生短暂的腱鞘炎、肌腱炎～表现为疼痛和触痛甚至红肿。关节液多为非炎症性改变。当复发性多软骨炎合并类风湿关节炎时～则可出现对称性侵蚀性畸形性关节炎。 5(呼吸系统病变:约半数患者累及喉、气管及支气管软骨。表现为声音嘶哑、刺激性咳嗽、呼吸困难和吸气性喘鸣。喉和气管炎症早期可有甲状软骨、环状软骨及气管软骨压痛。喉和会厌软骨炎症可导致上呼吸道塌陷～造成窒息～需急症行气管切开术。在疾病的晚期支气管也可发生类似病变～炎症、水肿及瘢痕形成可导致严重的局灶性或弥漫性的气道狭窄～气管切开术不能有效地纠正呼吸困难。由于呼吸道分泌物不能咳出～继发肺部感染～可导致患者死亡。 6(心血管病变:约30%的患者可累及心血管系统～表现为心肌炎、心内膜炎或心脏传导阻滞～主动脉瓣关闭不全～大、中、小血管炎。主动脉瓣关闭不全是常见而严重的心血管并发症～通常是由于主动脉炎症、主动脉瓣环和主动脉扩张所致～而非主动脉瓣膜病变～在主动脉瓣听诊区可闻及程度不同的舒张期杂音～其他的表现包括升主动脉、降主动脉动脉瘤～及其他大血管动脉瘤破裂引起猝死～此外～还可因血管炎而导致血栓形成～可累及降主及腹主动脉、锁骨下、脑内、肝、肠系膜及周围动脉～本病可伴发结节性多动脉炎、韦格纳肉芽肿及大动脉炎等。 7(血液系统异常:半数患者出现贫血、血小板减少～活动期的患者多有轻度正细胞正色素性贫血、白细胞增高～有些患者脾脏肿大～还可并发骨髓异常增生综合征,MDS,～表现为难治性贫血～红细胞系统、粒细胞系统、巨核细胞系统增生异常～少数患者发生溶血性贫血～可有黄疸、网织红细胞增加等表现。 8(皮肤损害:25%的患者有皮肤受损～皮损无特征性～形态多样～可表现为结节性红斑、紫癜、网状青斑、结节、皮肤角化、溢脓、色素沉着等～活检常呈白细胞破碎性血管炎的组织学改变～此外也可发生指,趾,甲生长迟缓、脱发及脂膜炎～口腔及生殖器黏膜溃疡～有些病例和白塞病重叠存在。 9(神经系统病变:少数患者可有中枢神经系统受损伤和周围神经受损的症状～如头痛、外展神经、面神经麻痹、癫痫、器质性脑病和痴呆～也可发生多发性单神经炎。 ht difference should not be greater than 10 g and knocks stamped mark. (See Figure 18) The coupling bolts apply lubricant, ant and lock washer to match. General requirements for coupling two symmetrical diameter bolt, nut and lock washer's total weigof nu essing, processing back bolt installation location cannot be changed, so according to bolt after the actual weight differencee to the coupling bolts in accordance with hinge holes corresponding to the diameter of the screw holes one by one after procboring, hinge bolt hole flange with coupling of vertical, not oblique. Dureaming hole in turn. Coupling bolts must be tight coupling is symmetrical diameter, in boring, reaming, two bolts out. Well -two coupling bolts before you can move the rotor, boring a goodld be marked. 6.4.5.4.2 coupling reamed and pairings is close to the diameter of bolt bearing good hinge holes, dressed with , typing gently with a small hammer, not too loose or too tight, packed the bolts with the corresponding seal screw hole shouicantss and so on. Well boring, hinge bolt hole flange with coupling of vertical, not oblique. Mounting bolts should be added lubrt allow any work that may affect the coupling Centre, such as irrigation, to cylinder connections to flood the condenser pipeapart from the special provisions in factory, does no coupling Centre shall be checked, the second poured concrete strength of more than 70%. In the process of reaming or boring,2ynamometric wrench or log tension tools. Three wheel rotor swing test coupling connections should be checked: taking leave,0.02mm. Tightening torque of the coupling bolts shall comply with the technical requirements manufacturers drawings, with a dhan 70%, 100% force, and check the coupling flutter and before connecting the circle gradually changes its value should be less t gently with copper home, not too loose or too tight. Bolt Assembly is finished, symmetrical tightening all bolts to 30%, 50%, d then 10(肾脏病变:肾脏受累的表现有显微镜下血尿、蛋白尿或管型尿～反复发作可导致严重肾炎和肾功能不全～肾动脉受累可发生高血压～肾脏活检有肾小球性肾炎的组织学证据～尿常规检测异常可能同肾脏损害有关～但也可因合并系统性血管炎引起。 二、实验室检查 1(血常规及血沉:大多数急性活动期患者有轻度正细胞正色素性贫血～及白细胞中度增高～血沉增速。 2(尿常规:少数患者有蛋白尿、血尿或管型尿～有时可出现类似于肾盂肾炎的改变～急性活动期尿中酸性黏多糖排泄增加～对诊断有参考价值。 3(血清学检查:约20%~25%的患者抗核抗体及类风湿因子阳性～少数患者梅毒血清学反应假阳性～或狼疮细胞阳性～总补体、C3、C4多正常～偶有升高～IgG、IgA在急性期可暂时性增高～间接免疫荧光法抗软骨细胞抗体阳性及抗II型胶原抗体阳性有助于复发性多软骨炎的诊断。 4(肾功能与脑脊液检查:肾功能异常及脑脊液细胞增多提示相关的血管炎。 5(X线检查:常有耳软骨钙化～喉断层摄影可见有气道狭窄～胸片显示肺不张、肺炎和程度不等的纤维化～气管支气管断层摄影可见气管、支气管普遍性狭窄～X线检查可见心脏扩大～并以左心扩大为主～有时也能显示主动脉弓扩大～升主动脉和降主动脉、鼻、气管和喉有钙化～关节X线检查示关节旁的骨密度降低～可有关节间隙狭窄～但无侵蚀性破坏～少数患者有脊柱后凸～腰椎和椎间盘有侵蚀及融合～骶髂关节狭窄及侵蚀～必要时行CT扫描检查。 6(纤维支气管镜检查及肺功能测定:纤维支气管镜检查可发现气管、支气管普遍狭窄～软骨环消失～黏膜增厚、充血水肿及坏死～内有肉芽肿样改变或黏膜苍白萎缩～由于气道狭窄或塌陷等改变～肺功能测定显示阻塞性通气障碍。 【概述】 复发性多软骨炎(relapsing polychondritis，RP)是一种较少见的炎性破坏性疾病，其特点是软骨组织复发性退化性炎症，表现为耳、鼻、喉、气管、眼、关节、心脏瓣膜等器官及血管等其他结缔组织受累。复发性多软骨炎的病因及发病机制目前仍不清楚。软骨基质受外伤、炎症、过敏等因素的影响暴露出抗原性，导致机体对软骨局部或有共同基质成分的组织(如巩膜、葡萄膜、玻璃体、视神经内膜及神经束膜、主动脉中层和内层的结缔组织、心瓣膜、心肌肌纤维膜、气管粘膜下基底膜、关节滑膜和肾小球及肾小管基底膜等)的免疫反应。复发性多软骨炎可与类风湿关节炎、系统性血管炎、系统性红斑狼疮以及其他结缔组织病并发。各年龄阶段均可发病，好发年龄为30~60岁，发病无性别倾向。病初常为急性炎症，经数周至数月好转，以后呈慢性反复发作。晚期因起 he circle gradually changes its value should be less than 0.02mm. Tightening torque of the coupling bolts shall comply with tnished, symmetrical tightening all bolts to 30%, 50%, 70%, 100% force, and check the coupling flutter and before connecting tis fi ee Figure 18) The coupling bolts apply lubricant, and then gently with copper home, not too loose or too tight. Bolt Assemblyical diameter bolt, nut and lock washer's total weight difference should not be greater than 10 g and knocks stamped mark. (Symmetraccording to bolt after the actual weight difference of nut and lock washer to match. General requirements for coupling two she diameter of the screw holes one by one after processing, processing back bolt installation location cannot be changed, so to t lt hole flange with coupling of vertical, not oblique. Due to the coupling bolts in accordance with hinge holes correspondingturn. Coupling bolts must be tight coupling is symmetrical diameter, in boring, reaming, two bolts out. Well boring, hinge bo reaming hole in-eter of bolt bearing good hinge holes, dressed with a good two coupling bolts before you can move the rotor, boringhe bolts with the corresponding seal screw hole should be marked. 6.4.5.4.2 coupling reamed and pairings is close to the diamked t, not oblique. Mounting bolts should be added lubricants, typing gently with a small hammer, not too loose or too tight, pacto cylinder connections to flood the condenser pipes and so on. Well boring, hinge bolt hole flange with coupling of vertical ,apart from the special provisions in factory, does not allow any work that may affect the coupling Centre, such as irrigation coupling Centre shall be checked, the second poured concrete strength of more than 70%. In the process of reaming or boring,3pling connections should be checked: taking leave,hnical requirements manufacturers drawings, with a dynamometric wrench or log tension tools. Three wheel rotor swing test couhe tec 支撑作用的软骨组织遭破坏，出现松软耳、鞍鼻以及嗅觉、视觉、听觉和前庭功能障碍。 【临床表现】 复发性多软骨炎可隐匿起病，也可骤发或病情突然加重。活动期可有发热、局部疼痛、疲乏无力、体重减轻和食欲不振等。常见临床表现如下: 1、耳软骨炎 耳廓软骨炎是最常见的临床表现。病变多局限于耳廓软骨部分，包括耳轮、耳屏，有时可侵犯外耳道，常对称性受累，但耳垂不受累。初期仅表现为耳廓红、肿、热、痛、有红斑结节，常在5~10天内自行消退，可反复发作，久之耳廓塌陷畸形，局部色素沉着。耳廓软骨炎可导致耳松软、变形、弹力减弱，出现结节，外耳道萎缩。外耳道狭窄、中耳炎症、咽鼓管阻塞可致传导性耳聋。后期可累及内耳，表现为听觉或前庭功能损伤。病变累及迷路可导致旋转性头晕、眼球震颤、共济失调、恶心及呕吐等。 2、鼻软骨炎 约3/4的患者有鼻软骨炎。在急性期表现为局部红肿，压痛，常突然发病，颇似蜂窝组织炎，数天后可缓解。反复发作可引起鼻软骨局限性塌陷，发展为鞍鼻畸形，甚者在发病1-2天内鼻梁可突然下陷。患者常有鼻塞、流涕、鼻衄、鼻粘膜糜烂及鼻硬结等。 3、眼部 眼部受累可单侧或者双侧，表现为突眼、巩膜外层炎、角膜炎或葡萄膜炎。巩膜炎反复发作可导致角膜外周变薄，甚至造成眼球穿孔。此外还可有球结膜水肿、结膜炎、角膜结膜炎、眼干燥、白内障、虹膜睫状体炎、眼外直肌麻痹等表现。视网膜病变如视网膜微小动脉瘤、出血、渗出，静脉闭塞、动脉栓塞也常有发生。视网膜血管炎或视神经炎可导致失明。随着病情的反复发作，患者常可同时有几种眼疾。 4、关节 复发性多软骨炎的关节损害特点是外周关节非侵蚀性非畸形性多关节炎。大小关节均可受累，呈非对称性分布，多为间歇性发作，慢性持续性者较少。肋软骨和胸锁关节以及骶髂关节也可受累。此外尚可发生短暂的腱鞘炎、肌腱炎，表现为疼痛和触痛甚至红肿。关节液多为非炎症性改变。当复发性多软骨炎合并类风湿关节炎时，则可出现对称性侵蚀性畸形性关节炎。 5、呼吸系统病变 约半数患者累及喉、气管及支气管软骨。表现为声音嘶哑，刺激性咳嗽，呼吸困难和吸气性喘鸣。喉和气管炎症早期可有甲状软骨、环状软骨及气管软骨压痛。喉和会厌软骨炎症可导致上呼吸道塌陷，造成窒息，需急症行气管切开术。在疾病的晚期支气管也可发生类似病变，炎症、水肿及瘢痕形成可导致严重的局灶性或弥漫性的气道狭窄、气管切开术不能有效地纠正呼吸困难。由于呼吸道分泌物不能咳出，继发肺部感染，可导致患者死亡。 7、心血管 约30%的患者可累及心血管系统，表现为心肌炎、心内膜炎、或心脏传导阻滞，主动脉瓣关闭不全，大、中、小血管炎。主动脉瓣关闭不全是常见而严重的心血管并发症，通常是由于主动脉炎症和主动脉瓣环 70%, 100% force, and check the coupling flutter and before connecting the circle gradually changes its value should be less t gently with copper home, not too loose or too tight. Bolt Assembly is finished, symmetrical tightening all bolts to 30%, 50%, d thenht difference should not be greater than 10 g and knocks stamped mark. (See Figure 18) The coupling bolts apply lubricant, ant and lock washer to match. General requirements for coupling two symmetrical diameter bolt, nut and lock washer's total weigof nu essing, processing back bolt installation location cannot be changed, so according to bolt after the actual weight differencee to the coupling bolts in accordance with hinge holes corresponding to the diameter of the screw holes one by one after procboring, hinge bolt hole flange with coupling of vertical, not oblique. Dureaming hole in turn. Coupling bolts must be tight coupling is symmetrical diameter, in boring, reaming, two bolts out. Well -two coupling bolts before you can move the rotor, boring a goodld be marked. 6.4.5.4.2 coupling reamed and pairings is close to the diameter of bolt bearing good hinge holes, dressed with , typing gently with a small hammer, not too loose or too tight, packed the bolts with the corresponding seal screw hole shouicantss and so on. Well boring, hinge bolt hole flange with coupling of vertical, not oblique. Mounting bolts should be added lubrt allow any work that may affect the coupling Centre, such as irrigation, to cylinder connections to flood the condenser pipeapart from the special provisions in factory, does no coupling Centre shall be checked, the second poured concrete strength of more than 70%. In the process of reaming or boring,4ynamometric wrench or log tension tools. Three wheel rotor swing test coupling connections should be checked: taking leave,0.02mm. Tightening torque of the coupling bolts shall comply with the technical requirements manufacturers drawings, with a dhan 和主动脉进行性扩张所致，而非主动脉瓣膜病变。在主动脉瓣听诊区可闻及程度不同的舒张期杂音。其他的表现包括升主动脉和降主动脉动脉瘤，大血管动脉瘤破裂可引起猝死。此外，还可出现因血管炎而导致的血栓形成，可累及降主及腹主动脉，锁骨下、脑内、肝、肠系膜及周围动脉。本病可伴发结节性多动脉炎、韦格纳肉芽肿及大动脉炎等。 8、血液系统 半数患者出现贫血、血小板减少。活动期的患者多有轻度正细胞正色素性贫血，白细胞增高。有些患者脾脏肿大，还可并发骨髓异常增生综合征(MDS)，表现为难治性贫血，红细胞系统、粒细胞系统、巨核细胞系统增生异常。少数发生溶血性贫血，可有黄疸、网织红细胞增加等表现。 9、皮肤 25%患者有皮肤受损。皮损无特征性，形态多样，可表现为结节性红斑、紫癜、网状青斑、结节、皮肤角化、溢脓、色素沉着等。活检常呈白细胞破碎性血管炎的组织学改变。此外也可发生指(趾)甲生长迟缓、脱发及脂膜炎，口腔及生殖器粘膜溃疡。有些病例和白塞病重叠存在。 10、神经系统病变 少数患者可有中枢神经系统受损伤和周围神经受损的症状，如头痛，外展神经、面神经麻痹、癫痫、器质性脑病和痴呆，也可发生多发性单神经炎。 11、肾脏病变 肾脏受累的表现有显微镜下血尿、蛋白尿或管型尿，反复发作可导致严重肾炎和肾功能不全。肾动脉受累可发生高血压。肾脏活检有肾小球性肾炎的组织学证据。尿常规检测异常可能同肾脏损害有关，但也可因合并系统性血管炎引起。 【实验室检查】 1、血常规及血沉:大多数急性活动期患者有轻度正细胞正色素性贫血，及白细胞中度增高，血沉增速。 2、尿常规:少数患者有蛋白尿、血尿或管型尿。有时可出现类似于肾盂肾炎的改变。急性活动期尿中酸性粘多糖排泄增加，对诊断有参考价值。 3、血清学检查:约20%~25%的患者抗核抗体及类风湿因子阳性。少数患者梅毒血清学反应假阳性，或狼疮细胞阳性。总补体、C3、C4多正常，偶有升高。IgA、IgG在急性期可暂时性增高。间接免疫荧光法抗软骨细胞抗体阳性及抗?型胶原抗体阳性有助于复发性多软骨炎的诊断。 4、肾功能异常及脑脊液细胞增多提示相关的血管炎。 5、,线检查:常有耳软骨钙化，喉断层摄影可见有气管狭窄。胸片显示肺不张、肺炎和程度不等的纤维化。气管支气管体层摄影可见气管、支气管普遍性狭窄。X线检查可见心脏扩大，并以左心扩大为主。有时也能显示主动脉弓进行性扩大，升主动脉和降主动脉、鼻、 气管和喉有钙化。关节X线检查示关节旁的骨密度降低，可有关节腔狭窄，但无侵蚀性破坏。少数患者有脊柱后凸，腰椎和椎间盘有侵蚀及融合，骶髂关节狭窄及侵蚀，必要时行CT扫描检查。 6、纤维支气管镜检查及肺功能测定:纤维支气管镜检查可发现气管、支气管普遍狭窄，软骨环消失，粘膜增厚、充血水肿及坏死，内有 lt hole flange with coupling of vertical, not oblique. Due to the coupling bolts in accordance with hinge holes correspondingturn. Coupling bolts must be tight coupling is symmetrical diameter, in boring, reaming, two bolts out. Well boring, hinge bo reaming hole in-eter of bolt bearing good hinge holes, dressed with a good two coupling bolts before you can move the rotor, boringhe bolts with the corresponding seal screw hole should be marked. 6.4.5.4.2 coupling reamed and pairings is close to the diamked t, not oblique. Mounting bolts should be added lubricants, typing gently with a small hammer, not too loose or too tight, pacto cylinder connections to flood the condenser pipes and so on. Well boring, hinge bolt hole flange with coupling of vertical ,apart from the special provisions in factory, does not allow any work that may affect the coupling Centre, such as irrigation coupling Centre shall be checked, the second poured concrete strength of more than 70%. In the process of reaming or boring,5pling connections should be checked: taking leave,hnical requirements manufacturers drawings, with a dynamometric wrench or log tension tools. Three wheel rotor swing test couhe teche circle gradually changes its value should be less than 0.02mm. Tightening torque of the coupling bolts shall comply with tnished, symmetrical tightening all bolts to 30%, 50%, 70%, 100% force, and check the coupling flutter and before connecting tis fi ee Figure 18) The coupling bolts apply lubricant, and then gently with copper home, not too loose or too tight. Bolt Assemblyical diameter bolt, nut and lock washer's total weight difference should not be greater than 10 g and knocks stamped mark. (Symmetraccording to bolt after the actual weight difference of nut and lock washer to match. General requirements for coupling two she diameter of the screw holes one by one after processing, processing back bolt installation location cannot be changed, so to t 肉芽肿样改变或粘膜苍白萎缩。由于气道狭窄或塌陷等改变肺功能测定显示阻塞性通气障碍。 【诊断要点】 根据典型的临床表现和实验室检查如有复发性多软骨炎的可能，可按1975年McAdam的标准诊断:?双耳软骨炎;?非侵蚀性多关节炎;?鼻软骨炎;?眼炎，包括结膜炎、角膜炎、巩膜炎、浅层巩膜炎及葡萄膜炎等;?喉和(或)气管软骨炎;? 耳蜗和(或)前庭受损，表现为听力丧失，耳鸣和眩晕。具有上述标准3条或3条以上者可以确诊，无需组织学证实。不足3条者需软骨活检证实诊断。 Damiani认为为早期诊断，应扩大McAdam的诊断标准，下述3条中的1条即可诊断:?符合McAdam诊断标准;?1条以上的McAdam征，加上病理证实，如做耳、鼻、 呼吸道软骨活检;?病变累及2个或2个以上的解剖部位，对糖皮质激素或氨苯砜有效。 当病变累及下列耳、鼻、喉、眼、气管软骨时，应与临床表现相类似的其他疾病相鉴别: 1、耳廓病变及外耳炎:应与局部外伤、冻疮、丹毒、慢性感染、 系统性红斑狼疮、痛风、霉菌性疾病、梅毒、麻风病鉴别。系统性血管炎或其他结缔组织病也可引起耳软骨炎，但双侧耳软骨炎者不多见。 2、鼻软骨炎:需要与韦格纳肉芽肿、淋巴样肉芽肿、 致死性中线肉芽肿、先天性梅毒、麻风、淋巴瘤、结核等引起的肉芽肿以及癌肿和淋巴肉瘤相鉴别。反复多次活检、病原菌的培养及血清学检查可有助鉴别。 3、眼炎:应注意与韦格纳肉芽肿、结节性多动脉炎、Cogan综合征、白塞病、原发性或继发性干燥综合征、血清阴性脊柱关节病等累及眼的全身性疾病相鉴别。根据这些疾病的全身表现和实验室检查特征不难与之区别。 4、气管支气管狭窄变形:应与感染性疾病、结节病、非感染性肉芽肿病、肿瘤、慢性阻塞性肺疾病、淀粉样变性等疾病鉴别，一般上述疾病经活组织检查明确诊断。复发性多软骨炎患者同时还有耳、鼻等软骨病变，可资与之鉴别。 5、主动脉炎和主动脉病变:应与梅毒、马凡综合征、Ehlers-Danlos 综合征、特发性纵隔囊肿坏死、血清阴性脊柱关节病并发的主动脉病变相鉴别。 6、肋软骨炎:需与良性胸廓综合征(如特发性、 外伤性肋软骨炎、Tietze综合征、肋胸软骨炎、剑突软骨综合征等)鉴别。上述这些疾病均无系统性临床表现，可与本病鉴别。 【治疗及原则】 1、一般治疗 急性发作期应卧床休息，视病情给予流质或半流质饮食，以免引起会厌和喉部疼痛。注意保持呼吸道通畅，预防窒息。烦躁不安者可适当用镇静剂，以保持充足的睡眠。 2、药物治疗 ?非甾类抗炎药:参照类风湿关节炎用药。 ?糖皮质激素:可抑制病变的急性发作，减少复发的频率及严重程度，用于病情较重者，开始剂量为0.5~1mg/kg/d，分次或晨起一次口服。对有喉、气管及支气管、眼、内耳等累及的急性重症患者，糖皮质激素 of nu essing, processing back bolt installation location cannot be changed, so according to bolt after the actual weight differencee to the coupling bolts in accordance with hinge holes corresponding to the diameter of the screw holes one by one after procboring, hinge bolt hole flange with coupling of vertical, not oblique. Dureaming hole in turn. Coupling bolts must be tight coupling is symmetrical diameter, in boring, reaming, two bolts out. Well -two coupling bolts before you can move the rotor, boring a goodld be marked. 6.4.5.4.2 coupling reamed and pairings is close to the diameter of bolt bearing good hinge holes, dressed with , typing gently with a small hammer, not too loose or too tight, packed the bolts with the corresponding seal screw hole shouicantss and so on. Well boring, hinge bolt hole flange with coupling of vertical, not oblique. Mounting bolts should be added lubrt allow any work that may affect the coupling Centre, such as irrigation, to cylinder connections to flood the condenser pipeapart from the special provisions in factory, does no coupling Centre shall be checked, the second poured concrete strength of more than 70%. In the process of reaming or boring,6ynamometric wrench or log tension tools. Three wheel rotor swing test coupling connections should be checked: taking leave,0.02mm. Tightening torque of the coupling bolts shall comply with the technical requirements manufacturers drawings, with a dhan 70%, 100% force, and check the coupling flutter and before connecting the circle gradually changes its value should be less t gently with copper home, not too loose or too tight. Bolt Assembly is finished, symmetrical tightening all bolts to 30%, 50%, d thenht difference should not be greater than 10 g and knocks stamped mark. (See Figure 18) The coupling bolts apply lubricant, ant and lock washer to match. General requirements for coupling two symmetrical diameter bolt, nut and lock washer's total weig 的剂量可酌情增加，甚至行甲基泼尼松龙冲击治疗。临床症状好转后，可逐渐减量，以最小维持剂量维持1-2年，或更长时间。 ?免疫抑制剂:可选用环磷酰胺、氨甲蝶呤、硫唑嘌呤等免疫抑制剂口服(详见类风湿关节炎及其他有关章节)。在使用免疫抑制剂时，应定期查血尿常规、肝肾功能以防止不良反应发生。 ?氨苯砜:氨苯砜在人体内可抑制补体的激活和淋巴细胞转化，也能抑制溶菌酶参予的软骨退行性变。剂量范围25-200mg/d，平均剂量为75mg/d，开始从小剂量试用，以后逐渐加量。因有蓄积作用，服药6日需停药1日，持续约6个月。氨苯砜主要副作用为恶心、嗜睡、溶血性贫血、药物性肝炎、及白细胞下降等。 3、对症治疗 (1)眼部症状 局部用泼尼松眼膏，或用氢化考的松眼药点眼。注意预防继发感染。当出现继发性白内障或青光眼时，给予相应治疗。 (2)对气管软骨塌陷引起重度呼吸困难的患者，应立即行气管切开术，必要时用人工呼吸机辅助通气，以取得进一步药物治疗的机会。对于软骨炎所至的局限性气管狭窄可行外科手术切除。积极预防和治疗肺部炎症，一旦发生肺部感染，应使用有效的抗生素。 (3)复发性多软骨炎患者因心瓣膜病变引起难治性心功能不全时,应使用强心剂和减轻心脏负荷的药物。若有条件可行瓣膜修补术或瓣膜成形术，以及主动脉瘤切除术。 【预后】 患者5年死亡率接近1/3，通常死于喉和气管软骨支持结构塌陷，或心血管病变(大动脉瘤、心脏瓣膜功能不全)或系统性血管炎。为降低死亡率，改善预后，应早期诊断和及时治疗。 通讯作者:李兴福 山东医科大学附属医院 邮编250012 复发性多软骨炎的诊断和治疗现状 中山大学附属第二医院风湿免疫科(510120) 莫颖倩 戴 冽 [关键词] 软骨 炎症 复发性多软骨炎 自身免疫性疾病 1 引 言 复发性多软骨炎(relapsing polychondritis，RP)是一种少见的累及多部位软骨的炎性破坏性疾病。该病可累及气道、外周关节和肋骨处的透明软骨，耳廓、鼻梁的弹性软骨，中轴关节的纤维软骨，以及存在于眼睛、心脏、血管、内耳等器官组织中的富含蛋白多糖的软骨共同基质。由于该病在我国较罕见，临床表现复杂多样，首发症状不典型，因而极易被误诊漏诊，临床需提高对该病的认识和重视。 2 发病机制 目前普遍认为，RP是一种自身免疫性疾病，其发病机制可能是各种原因引起的细胞和体液的免疫异常并攻击自身的软骨组织，导致含有软骨组织器官的结构 to t lt hole flange with coupling of vertical, not oblique. Due to the coupling bolts in accordance with hinge holes correspondingturn. Coupling bolts must be tight coupling is symmetrical diameter, in boring, reaming, two bolts out. Well boring, hinge bo reaming hole in-eter of bolt bearing good hinge holes, dressed with a good two coupling bolts before you can move the rotor, boringhe bolts with the corresponding seal screw hole should be marked. 6.4.5.4.2 coupling reamed and pairings is close to the diamked t, not oblique. Mounting bolts should be added lubricants, typing gently with a small hammer, not too loose or too tight, pacto cylinder connections to flood the condenser pipes and so on. Well boring, hinge bolt hole flange with coupling of vertical ,apart from the special provisions in factory, does not allow any work that may affect the coupling Centre, such as irrigation coupling Centre shall be checked, the second poured concrete strength of more than 70%. In the process of reaming or boring,7pling connections should be checked: taking leave,hnical requirements manufacturers drawings, with a dynamometric wrench or log tension tools. Three wheel rotor swing test couhe teche circle gradually changes its value should be less than 0.02mm. Tightening torque of the coupling bolts shall comply with tnished, symmetrical tightening all bolts to 30%, 50%, 70%, 100% force, and check the coupling flutter and before connecting tis fi ee Figure 18) The coupling bolts apply lubricant, and then gently with copper home, not too loose or too tight. Bolt Assemblyical diameter bolt, nut and lock washer's total weight difference should not be greater than 10 g and knocks stamped mark. (Symmetraccording to bolt after the actual weight difference of nut and lock washer to match. General requirements for coupling two she diameter of the screw holes one by one after processing, processing back bolt installation location cannot be changed, so 破坏，从而导致本病的发生。 研究发现，病变的耳软骨有免疫球蛋白和补体C3的沉积，急性期RP患者血清中有?型、?型和?型胶原的抗体，并且?型胶原抗体的滴度与症状的严重性 [1]相关。RP患者还可检测到针对软骨寡聚基质蛋白(cartilage oligomeric matrix protein，COMP)和Matrilin-1的抗体。Matrilin-1是一种主要表达于气管软骨的细胞外基质蛋白，其诱导的小鼠RP模型出现呼吸道窘迫和鼻软骨炎症，提示抗 [2]Matrilin-1抗体是一种组织特异性的致病性抗体并在RP的发病中起作用。研究还发现，RP发病与人类白细胞抗原(human leukocyte antigen，HLA)-DR4相关联，提示主要组织相容性复合体(majr histocompatibility complex，MHC)-?类抗原及T细胞也参与了RP的发病。 t and lock washer to match. General requirements for coupling two symmetrical diameter bolt, nut and lock washer's total weigof nu essing, processing back bolt installation location cannot be changed, so according to bolt after the actual weight differencee to the coupling bolts in accordance with hinge holes corresponding to the diameter of the screw holes one by one after procboring, hinge bolt hole flange with coupling of vertical, not oblique. Dureaming hole in turn. Coupling bolts must be tight coupling is symmetrical diameter, in boring, reaming, two bolts out. Well -two coupling bolts before you can move the rotor, boring a goodld be marked. 6.4.5.4.2 coupling reamed and pairings is close to the diameter of bolt bearing good hinge holes, dressed with , typing gently with a small hammer, not too loose or too tight, packed the bolts with the corresponding seal screw hole shouicantss and so on. Well boring, hinge bolt hole flange with coupling of vertical, not oblique. Mounting bolts should be added lubrt allow any work that may affect the coupling Centre, such as irrigation, to cylinder connections to flood the condenser pipeapart from the special provisions in factory, does no coupling Centre shall be checked, the second poured concrete strength of more than 70%. In the process of reaming or boring,8ynamometric wrench or log tension tools. Three wheel rotor swing test coupling connections should be checked: taking leave,0.02mm. Tightening torque of the coupling bolts shall comply with the technical requirements manufacturers drawings, with a dhan 70%, 100% force, and check the coupling flutter and before connecting the circle gradually changes its value should be less t gently with copper home, not too loose or too tight. Bolt Assembly is finished, symmetrical tightening all bolts to 30%, 50%, d thenht difference should not be greater than 10 g and knocks stamped mark. (See Figure 18) The coupling bolts apply lubricant, an