嗜酸性筋膜炎的临床和病理研究

发布时间：2018-07-06 15:09

本文选题：嗜酸性筋膜炎 + 系统性硬皮病　；参考：《山东大学》2015年硕士论文

【摘要】：背景及目的嗜酸性筋膜炎(Eosinophilic fasciitis, EF)是一种临床较为罕见的免疫变态反应性疾病,该病以肢体皮肤深筋膜硬皮病样病损为主要临床表现。EF病因及致病机制目前尚不明确。外周血嗜酸性粒细胞增多、高γ球蛋白血症及红细胞沉降率(Erythrocyte sedimentation rate, ESR)增高等实验室检查改变对疾病诊断具有提示意义。筋膜和肌肉活检呈现的典型病理特征对确诊该病至关重要,其主要包括深筋膜增厚及炎细胞浸润等改变。受累部位肌肉磁共振(Magnetic resonance imaging, MRI)是近年来推荐的影像学检查手段,T2加权成像显示深筋膜增厚且呈现高信号对EF诊断有重要意义,对筋膜和肌肉活检部位选择亦有提示意义。自1974年Shulman首次报道该病以来,学界尚未形成广泛认可的诊断标准。确诊该病需行筋膜和肌肉活检病理检查,同时结合病史、临床表现、实验室检查、影像学证据等综合判断分析。该病因少见、临床表现多样及各种检查结果的非特异性,易误诊为其他肌肉病变。特别地,EF与早期系统性硬皮病(Systemic scleroderma, SSc)的临床表现、实验室检查及影像学检查均有相似之处,故在临床实践中需谨慎鉴别。本研究详细报道3例EF患者和1例早期SSc患者的临床资料,结合国内外相关文献,旨在进一步归纳总结EF的临床特征、病理改变及治疗方案,并着力讨论其与早期SSc的鉴别诊断。资料与方法本研究纳入自2013年1月至2014年12月就诊于山东大学齐鲁医院神经内科3例EF患者,详细采集患者病史,并行神经系统体格检查、实验室检查、影像学检查(胸部CT [Computed tomography, CT]平扫、心脏彩超和肌肉MRI平扫)、肌电图检查(Electromyography, EMG)及筋膜和肌肉活检病理检查以明确诊断。EF诊断标准参考2014年Fernandez等人提出的诊断细则。明确诊断后患者行系统治疗并定期随访进行病情监测、各项指标复查和治疗方案的调整优化。3例患者均随访至半年以上。同时收集1例与EF临床症状及实验室指标相似的早期SSc患者的临床资料以讨论鉴别诊断。结果(1)3例患者均为男性,平均发病年龄为24岁。2例患者发病前有过度疲劳史,1例患者发病前有外伤骨折史。3例患者症状均起自小腿,表现为双侧小腿对称性肿胀及按压或活动时肌肉疼痛,水肿消退后双侧小腿皮肤肌肉僵硬,逐渐向双上肢蔓延,出现双侧前臂皮肤肌肉发硬。关节活动受限以双侧腕关节、掌指关节及膝关节多见。神经系统体格检查示3例患者均出现Pray征,1例患者有Groove征,3例患者均未见Peau d'orange 征;1例患者受累肌肉可见肌力5-级,3例患者受累肢体腱反射均可见对称性减低或消失,余体格检查无明显变化。(2)实验室检验结果显示3例患者未治疗前均有外周血嗜酸性粒细胞增多及炎性指标如ESR、C反应蛋白(C reactive protein, CRP)及血清免疫球蛋白E (Immunoglobulin E, Ig E)增高。1例患者抗核抗体(Antinuclear antibody,ANA)低滴度阳性,其余检验结果均未见明显异常。(3)影像学检查示3例患者胸部平扫及心脏彩超均未见明显异常。1例患者行双侧小腿肌肉MRI平扫示双小腿皮肤脂肪层下区异常信号。(4)筋膜和肌肉活检结果示3例患者均有深筋膜增厚,其内大灶性炎细胞浸润,可见淋巴细胞、组织细胞、浆细胞及嗜酸性粒细胞。2例见炎细胞侵犯肌纤维,发现坏死吞噬及再生肌纤维。Ⅰ类主要组织相容性抗原(Majorhistocompatibility complex-Ⅰ, MHC-Ⅰ)免疫组织化学染色示患者深筋膜及肌纤维膜着色较正常对照明显增强。(5)EMG检查与筋膜和肌肉活检结果一致,2例伴发肌炎的患者EMG呈肌源性损害,另1例未见明显异常。(6)3例患者均接受糖皮质激素+免疫抑制剂治疗,其中初期单纯使用糖皮质激素可使实验室各项指标恢复正常,但临床症状改善不明显且不能阻止病情进展,加用免疫抑制剂总量达一定剂量后病情明显好转。伴发肌炎的EF患者症状改善较单纯EF患者缓慢,疗程较长。(7)EF与早期SSc的鉴别诊断：二者临床表现及实验室检查结果均有相似之处,若患者出现雷诺现象、手指肿胀、ANA高滴度阳性及多系统脏器受累则须考虑SSc可能性。结论(1)EF发病率较低,发病前可有过度疲劳及外伤等诱因,患者出现双侧肢体对称性皮肤肌肉变硬及关节活动受限等特征性临床表现；需注意该病患者有无雷诺现象、肢端硬化及其他系统受累表现,以与SSc进行鉴别。(2)实验室检查除外周血嗜酸性粒细胞增高外可表现为炎症综合症,即炎症标志物如ESR、CRP及IgE均增高。肌肉MRI如发现筋膜增厚且T2加权成像高信号可协助诊断。肌肉及筋膜活检为该病诊断的金标准,深筋膜增厚,可见大量炎细胞浸润,偶见嗜酸性粒细胞。(3)EF可伴发肌炎,由筋膜和肌肉活检及EMG检查可证实,伴发肌炎者临床表现与单纯EF者可无明显差别,体格检查肌力可无明显减退,血清肌酶可不升高；但伴发肌炎者治疗过程中症状缓解较单纯筋膜炎者慢,病程及疗程较长。(4)糖皮质激素为EF药物治疗之基石,单纯糖皮质激素治疗疗效不佳者,推荐联合使用免疫抑制剂。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
【学位授予单位】：山东大学
【学位级别】：硕士
【学位授予年份】：2015
【分类号】：R593.2

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