本文選題：信號識別顆粒 + 肌炎特異抗體��； 參考：《復(fù)旦大學》2014年博士論文

【摘要】：目的分析中國南方人群的肌炎抗體譜,觀察并分析信號識別顆粒(signal recognition particle,SRP)抗體相關(guān)壞死性肌病患者的臨床及肌肉病理表現(xiàn)。方法收集2012年12月至2014年2月就診于我院的行肌炎抗體譜檢測并有明確診斷信息的病例114例,計算肌炎抗體對炎性肌病的靈敏度、特異度及炎性肌病中各肌炎抗體的陽性率。收集其中SRP抗體陽性的炎性肌病共26例患者的臨床資料,并隨訪其治療及預(yù)后,對其中22例患者行肌肉活檢,進行蘇木精-伊紅染色,CD4、CD8、MHC-I、C5b-9、KP-1免疫組織化學染色。分組統(tǒng)計比較急性組(癥狀達高峰時間≤6m)亞急性組(6m癥狀達高峰時間≤12m)和慢性組(癥狀達高峰時間12m)患者的臨床表現(xiàn)和肌肉病理表現(xiàn),分組統(tǒng)計比較病程不同階段的肌肉病理表現(xiàn)。結(jié)果肌炎抗體對炎性肌病的靈敏度為74%,特異度為88%；肌炎特異抗體的靈敏度為52%,特異度為94%；肌炎相關(guān)抗體的靈敏度為52%,特異度為94%；SRP抗體(28%)為最常見的肌炎特異抗體,其次為Mi-2抗體(8%)和Jo-1抗體(8%)；Ro-52抗體(41.9%)為最常見的肌炎相關(guān)抗體。26例SRP抗體相關(guān)壞死性肌病患者性別比女：男=16：10,發(fā)病年齡最小16歲,最大62歲,平均42.9+12.1歲。發(fā)病季節(jié)未見明顯集中趨勢；癥狀達高峰的中位時間6.0個月(0.5-24.0個月),最高肌酸激酶(creatine kinase, CK)中位值8704.0U/L(1349.0U/L-14620.0U/L),最差MMT8評分平均53.3+9.9；可見肌肉萎縮(69.2%)、吞咽或構(gòu)音障礙(46.2%)、肌肉酸痛(46.2%)、肺部間質(zhì)性病變(50%)、心電圖異常(33.3%)、心超異常(37.5%)；22例患者采用兩種或以上治療手段,治療有效21例(80.8%),難治4例(15.4%),依賴1例(3.8%),男性治療效果顯著差于女性(p=0.014),復(fù)發(fā)或復(fù)燃8例(30.8%)；慢性組病例發(fā)病年齡高于急性組(P=0.011)和亞急性組(P=0.024), CK max低于急性組(P=0.014)和亞急性組(P=0.006),最差MMT8評分有高于急性組(P=0.074)和亞急性組(P＝0.155)的趨勢,亞急性組的治療效果差于急性組(P=0.005)；按發(fā)展過程可將多數(shù)患者的病程分為五個階段：Ⅰ期(臨床前期)、Ⅱ期(發(fā)病期)、Ⅲ期(治療早期)、Ⅳ期(治療晚期)及V期(緩解期)。肌肉病理總體表現(xiàn)為程度不等的肌纖維的散在變性、壞死和新生、肌纖維大小不等、肌束內(nèi)結(jié)締組織增多、炎性浸潤、MHC-Ⅰ表達上調(diào)及C5b-9沉積；病程Ⅲ、Ⅳ期患者肌纖維新生、肌纖維大小不等、肌束內(nèi)結(jié)締組織增多、C5b-9在非壞死肌纖維膜的沉積顯著重于Ⅰ、Ⅱ期(p0.05),肌纖維壞死有輕于Ⅰ、Ⅱ期的傾向(p=0.385), MHC-I表達上調(diào)有重于Ⅰ、Ⅱ期的傾向(p=0.093)。隨訪1例患者Ⅱ、Ⅳ、Ⅴ期分別的SRP抗體結(jié)果均為3+,未見該抗體轉(zhuǎn)陰。結(jié)論肌炎抗體譜檢測在中國南方人群中對炎性肌病有較高的靈敏度和特異度,SRP抗體在中國南方炎性肌病群體中陽性率為28%。SRP抗體相關(guān)壞死性肌病的主要臨床表現(xiàn)為迅速進展的四肢近端肌無力,肌無力癥狀重,CK升高明顯,肌肉萎縮、消瘦和吞咽或構(gòu)音障礙較突出；多數(shù)患者需要2-3種治療手段,對治療多有效但易反復(fù),女性患者治療效果好于男性；按癥狀達高峰時間所分的慢性組發(fā)病年齡大,肌無力癥狀和CK升高較輕,亞急性組治療效果較差；肌肉病理表現(xiàn)為程度不等的肌纖維的散在變性、壞死和新生、肌纖維大小不等、肌束內(nèi)結(jié)締組織增多、炎性浸潤、MHC-I表達上調(diào)及C5b-9沉積；隨病程發(fā)展到后期,肌纖維新生、肌纖維大小不等、肌束內(nèi)結(jié)締組織增多、MHC-I表達上調(diào)和C5b-9在非壞死肌纖維膜的沉積更加明顯,而肌纖維散在壞死則開始減少,提示臨床病程不同階段的病理基礎(chǔ)。
[Abstract]:Objective to analyze the antibody spectrum of myositis in the southern Chinese population and to observe and analyze the clinical and muscular pathological features of signal recognition particle (SRP) antibody related necrotizing myopathy. Methods 114 cases of anti somatic detection of myositis in our hospital from December 2012 to February 2014 with definite diagnostic information were collected. The sensitivity and specificity of myositis antibody against inflammatory myopathy and the positive rate of antibodies in myositis in myositis were calculated. The clinical data of 26 patients with inflammatory myopathy with positive SRP antibody were collected, and the treatment and prognosis were followed up. 22 of them were performed by muscle biopsy, hematoxylin eosin staining, CD4, CD8, MHC-I, C5b-9, KP-1 immunology Group statistics compared the clinical manifestations and muscle pathological features of acute group (symptom peak time < 6m) subacute group (6m symptom peak time < 12m) and chronic group (symptom peak time 12m), and compare the muscle pathological features at different stages of disease course. Results the sensitivity of myositis antibody to inflammatory myopathy For 74%, the specificity was 88%, the sensitivity of the myositis specific antibody was 52%, the specificity was 94%, the sensitivity of the myositis related antibodies was 52%, the specificity was 94%; the SRP antibody (28%) was the most common myositis specific antibody, followed by the Mi-2 antibody (8%) and the Jo-1 antibody (8%), and the Ro-52 anti body (41.9%) was the most common myositis related antibody in.26 case SRP antibody correlation. The sex ratio of the patients with necrotic myopathy: male =16:10, the age of onset was 16 years old, the maximum 62 years old, the average 42.9+12.1 years old. There was no obvious concentration trend in the onset season; the median time of the peak was 6 months (0.5-24.0 months), the highest level of creatine kinase (creatine kinase, CK) was 8704.0U/L (1349.0U/L-14620.0U/L), and the worst MMT8 score was flat. Both 53.3+9.9, muscle atrophy (69.2%), dysphagia or dysarthria (46.2%), muscle soreness (46.2%), pulmonary interstitial lesions (50%), abnormal electrocardiogram (33.3%) and cardiac abnormal (37.5%); 22 patients were treated with two or more treatments, 21 (80.8%), 4 (15.4%), 1 cases (15.4%), and 1 cases (80.8%)), and the effect of male treatment was significantly worse than that of women. 8 cases (p=0.014), relapse or reignition (30.8%); chronic group cases were higher than acute group (P=0.011) and subacute group (P=0.024), CK Max was lower than acute group (P=0.014) and subacute group (P=0.006), the worst MMT8 score was higher than that in acute group (P=0.074) and subacute group (P = 0.155), and the treatment effect of subacute group was worse than that of acute group (P=0 group) .005); according to the development process, the course of most patients can be divided into five stages: phase I (preclinical), stage II (stage of disease), stage III (early treatment), stage IV (late treatment) and V phase (remission stage). Muscle pathology in general expression of muscle fibers in varying degrees is denatured, necrotic and new, muscle fiber size is different, and connective tissue within the muscle bundle increases. More, inflammatory infiltration, MHC- I expression up and C5b-9 deposition, course III, stage III, stage IV, muscle fiber newborn, muscle fiber size, the increase of connective tissue in the muscle, C5b-9 in the non necrotic muscle fibrous membrane deposition significantly more than I, stage II (P0.05), muscle fiber necrosis is lighter than the tendency of stage I, stage II (p=0.385), MHC-I expression up-regulation is heavier than I, II stage II stage heavier than stage I, stage II stage (stage II) The tendency (p=0.093). The results of the follow-up of 1 patients with SRP antibodies in stage II, IV and V were 3+, and the antibody was detected in the southern Chinese population with high sensitivity and specificity. The positive rate of SRP antibody in the southern inflammatory myopathy group in southern China was 28%.SRP antibody related necrotizing myopathy. The main clinical manifestations are the proximal muscle weakness of the extremities, muscle weakness, muscle weakness, muscle atrophy, muscle atrophy, emaciation and dysphagia or dysarthria. Most patients need 2-3 kinds of treatment, more effective but easy to repeat, and the treatment effect of women is better than that of men; chronic groups divided according to the peak time of symptoms The age was large, myasthenia symptoms and CK increased slightly, and the subacute group was less effective. The muscle pathological manifestations of the muscle fibers were diffuse, necrotic and new, muscle fiber size, connective tissue in the myosus, inflammatory infiltration, MHC-I expression up-regulated and C5b-9 deposition; with the development of the course, muscle fiber newborn, muscle The fiber size was different, the connective tissue in the myocutaneous bundle increased, the expression of MHC-I was up and the deposition of C5b-9 in the non necrotic muscle fibrous membrane was more obvious, while the muscle fiber scattered in the necrosis began to decrease, suggesting the pathological basis of the different stages of the clinical course.
【學位授予單位】：復(fù)旦大學
【學位級別】：博士
【學位授予年份】：2014
【分類號】：R746.1

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