本文選題：嗜酸性筋膜炎 + 系統(tǒng)性硬皮病��； 參考：《山東大學(xué)》2015年碩士論文

【摘要】：背景及目的嗜酸性筋膜炎(Eosinophilic fasciitis, EF)是一種臨床較為罕見的免疫變態(tài)反應(yīng)性疾病,該病以肢體皮膚深筋膜硬皮病樣病損為主要臨床表現(xiàn)。EF病因及致病機制目前尚不明確。外周血嗜酸性粒細(xì)胞增多、高γ球蛋白血癥及紅細(xì)胞沉降率(Erythrocyte sedimentation rate, ESR)增高等實驗室檢查改變對疾病診斷具有提示意義。筋膜和肌肉活檢呈現(xiàn)的典型病理特征對確診該病至關(guān)重要,其主要包括深筋膜增厚及炎細(xì)胞浸潤等改變。受累部位肌肉磁共振(Magnetic resonance imaging, MRI)是近年來推薦的影像學(xué)檢查手段,T2加權(quán)成像顯示深筋膜增厚且呈現(xiàn)高信號對EF診斷有重要意義,對筋膜和肌肉活檢部位選擇亦有提示意義。自1974年Shulman首次報道該病以來,學(xué)界尚未形成廣泛認(rèn)可的診斷標(biāo)準(zhǔn)。確診該病需行筋膜和肌肉活檢病理檢查,同時結(jié)合病史、臨床表現(xiàn)、實驗室檢查、影像學(xué)證據(jù)等綜合判斷分析。該病因少見、臨床表現(xiàn)多樣及各種檢查結(jié)果的非特異性,易誤診為其他肌肉病變。特別地,EF與早期系統(tǒng)性硬皮病(Systemic scleroderma, SSc)的臨床表現(xiàn)、實驗室檢查及影像學(xué)檢查均有相似之處,故在臨床實踐中需謹(jǐn)慎鑒別。本研究詳細(xì)報道3例EF患者和1例早期SSc患者的臨床資料,結(jié)合國內(nèi)外相關(guān)文獻(xiàn),旨在進(jìn)一步歸納總結(jié)EF的臨床特征、病理改變及治療方案,并著力討論其與早期SSc的鑒別診斷。資料與方法本研究納入自2013年1月至2014年12月就診于山東大學(xué)齊魯醫(yī)院神經(jīng)內(nèi)科3例EF患者,詳細(xì)采集患者病史,并行神經(jīng)系統(tǒng)體格檢查、實驗室檢查、影像學(xué)檢查(胸部CT [Computed tomography, CT]平掃、心臟彩超和肌肉MRI平掃)、肌電圖檢查(Electromyography, EMG)及筋膜和肌肉活檢病理檢查以明確診斷。EF診斷標(biāo)準(zhǔn)參考2014年Fernandez等人提出的診斷細(xì)則。明確診斷后患者行系統(tǒng)治療并定期隨訪進(jìn)行病情監(jiān)測、各項指標(biāo)復(fù)查和治療方案的調(diào)整優(yōu)化。3例患者均隨訪至半年以上。同時收集1例與EF臨床癥狀及實驗室指標(biāo)相似的早期SSc患者的臨床資料以討論鑒別診斷。結(jié)果(1)3例患者均為男性,平均發(fā)病年齡為24歲。2例患者發(fā)病前有過度疲勞史,1例患者發(fā)病前有外傷骨折史。3例患者癥狀均起自小腿,表現(xiàn)為雙側(cè)小腿對稱性腫脹及按壓或活動時肌肉疼痛,水腫消退后雙側(cè)小腿皮膚肌肉僵硬,逐漸向雙上肢蔓延,出現(xiàn)雙側(cè)前臂皮膚肌肉發(fā)硬。關(guān)節(jié)活動受限以雙側(cè)腕關(guān)節(jié)、掌指關(guān)節(jié)及膝關(guān)節(jié)多見。神經(jīng)系統(tǒng)體格檢查示3例患者均出現(xiàn)Pray征,1例患者有Groove征,3例患者均未見Peau d'orange 征;1例患者受累肌肉可見肌力5-級,3例患者受累肢體腱反射均可見對稱性減低或消失,余體格檢查無明顯變化。(2)實驗室檢驗結(jié)果顯示3例患者未治療前均有外周血嗜酸性粒細(xì)胞增多及炎性指標(biāo)如ESR、C反應(yīng)蛋白(C reactive protein, CRP)及血清免疫球蛋白E (Immunoglobulin E, Ig E)增高。1例患者抗核抗體(Antinuclear antibody,ANA)低滴度陽性,其余檢驗結(jié)果均未見明顯異常。(3)影像學(xué)檢查示3例患者胸部平掃及心臟彩超均未見明顯異常。1例患者行雙側(cè)小腿肌肉MRI平掃示雙小腿皮膚脂肪層下區(qū)異常信號。(4)筋膜和肌肉活檢結(jié)果示3例患者均有深筋膜增厚,其內(nèi)大灶性炎細(xì)胞浸潤,可見淋巴細(xì)胞、組織細(xì)胞、漿細(xì)胞及嗜酸性粒細(xì)胞。2例見炎細(xì)胞侵犯肌纖維,發(fā)現(xiàn)壞死吞噬及再生肌纖維。Ⅰ類主要組織相容性抗原(Majorhistocompatibility complex-Ⅰ, MHC-Ⅰ)免疫組織化學(xué)染色示患者深筋膜及肌纖維膜著色較正常對照明顯增強。(5)EMG檢查與筋膜和肌肉活檢結(jié)果一致,2例伴發(fā)肌炎的患者EMG呈肌源性損害,另1例未見明顯異常。(6)3例患者均接受糖皮質(zhì)激素+免疫抑制劑治療,其中初期單純使用糖皮質(zhì)激素可使實驗室各項指標(biāo)恢復(fù)正常,但臨床癥狀改善不明顯且不能阻止病情進(jìn)展,加用免疫抑制劑總量達(dá)一定劑量后病情明顯好轉(zhuǎn)。伴發(fā)肌炎的EF患者癥狀改善較單純EF患者緩慢,療程較長。(7)EF與早期SSc的鑒別診斷：二者臨床表現(xiàn)及實驗室檢查結(jié)果均有相似之處,若患者出現(xiàn)雷諾現(xiàn)象、手指腫脹、ANA高滴度陽性及多系統(tǒng)臟器受累則須考慮SSc可能性。結(jié)論(1)EF發(fā)病率較低,發(fā)病前可有過度疲勞及外傷等誘因,患者出現(xiàn)雙側(cè)肢體對稱性皮膚肌肉變硬及關(guān)節(jié)活動受限等特征性臨床表現(xiàn)；需注意該病患者有無雷諾現(xiàn)象、肢端硬化及其他系統(tǒng)受累表現(xiàn),以與SSc進(jìn)行鑒別。(2)實驗室檢查除外周血嗜酸性粒細(xì)胞增高外可表現(xiàn)為炎癥綜合癥,即炎癥標(biāo)志物如ESR、CRP及IgE均增高。肌肉MRI如發(fā)現(xiàn)筋膜增厚且T2加權(quán)成像高信號可協(xié)助診斷。肌肉及筋膜活檢為該病診斷的金標(biāo)準(zhǔn),深筋膜增厚,可見大量炎細(xì)胞浸潤,偶見嗜酸性粒細(xì)胞。(3)EF可伴發(fā)肌炎,由筋膜和肌肉活檢及EMG檢查可證實,伴發(fā)肌炎者臨床表現(xiàn)與單純EF者可無明顯差別,體格檢查肌力可無明顯減退,血清肌酶可不升高；但伴發(fā)肌炎者治療過程中癥狀緩解較單純筋膜炎者慢,病程及療程較長。(4)糖皮質(zhì)激素為EF藥物治療之基石,單純糖皮質(zhì)激素治療療效不佳者,推薦聯(lián)合使用免疫抑制劑。CLINICAL AND PATHOLOGICAL RESEARCH FOR
[Abstract]:Background and objective Eosinophilic fasciitis (EF) is a rare and clinically rare immune allergic disease. The disease is the main clinical manifestation of the skin deep fascia like lesion of the limb skin. The etiology and pathogenesis of.EF are not yet clear. Peripheral blood eosinophilic granulocytosis, high gamma globulinemia and red blood cells Changes in Erythrocyte sedimentation rate, ESR, and so on are suggestive of the diagnosis of the disease. The typical pathological features of the fascia and muscle biopsy are crucial to the diagnosis of the disease, mainly including the changes in the deep fascia and inflammatory cell infiltration. The muscle magnetic resonance (Magnetic resonance imaging) of the affected area (Magnetic resonance) MRI) is a recommended method of imaging examination in recent years. T2 weighted imaging shows that the thickening of the deep fascia and high signal are important for the diagnosis of EF. It is also suggestive of the selection of the fascia and muscle biopsy site. Since the first report of the disease in 1974, the academic community has not yet formed a widely recognized diagnostic standard. The diagnosis of the disease requires fascia and the diagnosis of the disease. Pathological examination of muscle biopsy, combined with a comprehensive analysis of the history, clinical manifestation, laboratory examination, and imaging evidence. The etiology is rare, the clinical manifestations and various examination results are nonspecific, and are easily misdiagnosed as other muscular lesions. In particular, the clinical manifestations of EF and early systemic scleroderma (Systemic scleroderma, SSc) are true. The clinical data of 3 EF patients and 1 early SSc patients were reported in detail, combined with relevant literature at home and abroad, the purpose of this study was to further summarize the clinical features, pathological changes and treatment schemes of EF, and to discuss their identification with early SSc. Diagnosis. Data and methods the study was taken from January 2013 to December 2014 in 3 patients with EF in the Department of Neurology, Qilu Hospital, Shandong University. The history of the patients was collected in detail, the physical examination of the nervous system, the laboratory examination, the imaging examination (chest CT [Computed tomography, CT] plain scan, cardiac color Doppler and muscle MRI plain scan), and electromyography examination Electromyography (EMG) and the pathological examination of fascia and muscle biopsy in order to make a clear diagnosis of the diagnostic criteria of.EF, refer to the diagnostic rules put forward by Fernandez et al. In 2014. After the diagnosis, the patients were treated with systematic treatment and regularly followed up for the monitoring of the disease. All the indexes rechecked and the treatment plan was adjusted and optimized. All the patients with.3 were followed up to more than half a year. The clinical data of 1 early SSc patients similar to EF clinical symptoms and laboratory indicators were collected to discuss the differential diagnosis. Results (1) 3 patients were male, the average age of onset was 24 years old and.2 patients had overfatigue history before onset. The symptoms of 1 patients with history of traumatic fracture before onset were all from the calf, showing bilateral calves. Symmetrically swollen and muscle pain during pressure or activity, the skin and muscles of the bilateral calves were stiff after the edema subsided, and gradually spread to the double upper limbs. Bilateral forearm skin and muscles were hard. Joint activities were limited by bilateral wrist joints, metacarpophalangeal joints and knee joints. 3 patients had Pray signs and 1 patients had Groove. There was no Peau d'orange sign in 3 patients; 1 cases showed muscle strength 5- level in the affected muscles. There was no obvious change in the symmetry of the tendon reflex in 3 patients. (2) the results of laboratory test showed that there were increased eosinophils in peripheral blood and inflammatory indexes such as ESR, C reaction before the treatment of 3 patients. C reactive protein, CRP) and serum immunoglobulin E (Immunoglobulin E, Ig E) increased the low titer of the anti nuclear antibody (Antinuclear antibody), and the other test results were not obvious abnormality. (3) imaging examination showed that both chest plain and heart color Doppler ultrasound showed no obvious abnormalities in 3 cases of patients with bilateral calf. The muscle MRI scan showed abnormal signal of subcutaneous fat layer in the skin of double calves. (4) the fascia and muscle biopsy showed that 3 patients had deep fascia thickening and infiltration of large focal inflammatory cells in them. There were.2 cases of lymphocytes, tissue cells, plasma cells and eosinophils, which showed inflammatory cells invading muscle fibers, necrophagocytic phagocytosis and regenerative muscle fibers. The immunohistochemical staining of Majorhistocompatibility complex- I (MHC- I) showed that the deep fascia and myosmus coloring of the patients were significantly enhanced. (5) the EMG examination was consistent with the results of fascia and muscle biopsy. In 2 patients with myositis, EMG showed myogenic damage and no obvious abnormalities were found in 1 cases. (6) 3 patients were all patients. With glucocorticoid and immunosuppressive therapy, the initial use of glucocorticoid can make the laboratory indexes back to normal, but the improvement of clinical symptoms is not obvious and can not prevent the progress of the disease. After adding the total amount of immunosuppressants to a certain dose, the condition of the disease is obviously improved. The symptoms of EF patients with myositis are better than those of the simple EF patients. Slow, long course of treatment. (7) differential diagnosis between EF and early SSc: the clinical manifestations and laboratory results of the two were similar. If the patient appeared Reynolds phenomenon, finger swelling, ANA high titer positive and multi system organ involvement, the possibility of SSc should be considered. Conclusion (1) the incidence of EF is low, and the causes of excessive fatigue and trauma can be induced before the onset of the disease. The patient has the characteristic clinical manifestations of bilateral symmetrical skin and muscle rigidity and limited joint activity. It should be noted that the patient has Renault phenomenon, acromegalosclerosis and other systemic involvement, and is identified with SSc. (2) laboratory examination, except for the peripheral blood eosinophilic granulocyte increase, can be shown as inflammatory syndrome, that is, inflammation markers. ESR, CRP and IgE were increased. Muscle MRI was found to be thickened and T2 weighted imaging could help diagnosis. Muscle and fascia biopsy was the gold standard for the diagnosis of the disease. Deep fascia was thickened, a large number of inflammatory cells were infiltrated, and eosinophilic granulocytes were seen occasionally. (3) EF can be associated with myositis, which were confirmed by fascia and muscle biopsy and EMG examination. There is no obvious difference between the clinical manifestations of the inflammatory patients and the simple EF, the physical strength of the physical examination can not be reduced obviously, the serum muscle enzymes can not be raised, but the symptoms of the patients with myositis are slower than those of the simple fasciitis, and the course and course of treatment are longer. (4) the corticosteroid is the cornerstone of the treatment of EF, and the simple glucocorticoid treatment is not good for those who have poor curative effect. Recommended use of immunosuppressive agents.CLINICAL AND PATHOLOGICAL RESEARCH FOR
【學(xué)位授予單位】：山東大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2015
【分類號】：R593.2

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