48例复发性多软骨炎临床特点及疗效分析

发布时间：2018-01-12 05:23

  本文关键词：48例复发性多软骨炎临床特点及疗效分析　出处：《郑州大学》2017年硕士论文　论文类型：学位论文

  更多相关文章： 复发性多软骨炎 累及呼吸道 误诊率 疗效

【摘要】：背景复发性多软骨炎(relapsing polychondritis,RP)是一种以软骨组织复发性退化性炎症为特点的较为少见的疾病,其具体发病原因及机制尚不清楚,目前认为与机体的自身免疫反应有密切关系。机体受外界刺激如炎症、外伤或过敏等因素的影响,作用于软骨基质,使其暴露出抗原,致使机体对软骨组织或具有相同软骨基质成分的组织结构(如眼部的结膜、角膜、巩膜、葡萄膜、视神经内膜、玻璃体;气管粘膜下的基底膜;关节滑膜;心脏瓣膜;主动脉中层和内层结缔组织;肾小球及肾小管基底膜等)造成破坏。可单个器官或组织病变,亦可累及多个器官或组织,常累及的器官或组织有眼、耳、鼻、喉、气管及支气管、关节、心脏瓣膜和肾等。约半数患者可累及呼吸系统,或以首发症状或是病程中出现,慢性进展,反复发作,易被误诊或漏诊。该病发病率低,发病机制尚不明确,临床表现不典型,病变特征不突出,加之缺乏特异性的血清学指标,影像学检查不易被发现,其软骨组织病理活检是诊断该病的金标准,但因获取活检组织不易获得,需创伤性操作,且关于该病的文献较少等一系列因素,导致临床医务工作者对该病认识上的缺乏,以上种种原因给确诊此病增加了难度,进而延长患者的住院时间、增大误诊率或漏诊率,增加患者及社会的经济负担。因此,了解该病的流行病学特征、重视该病的临床表现、深入探究该病的发病机制、研究其特异性的实验室指标、提高对该病影像学特点的认识、熟悉该病的诊断标准、探究行之有效的治疗方案、加强患者预后的随访工作势在必行。目的探讨复发性多软骨炎的流行病学特征、临床表现、实验室及影像学检查、其他特异性检查(肺功能、气管镜)、合并症、治疗方法及疗效。探讨呼吸道受累组和呼吸道未受累组在性别、发病年龄、首次误诊率、血清学指标、确诊时间、合并症及转归情况是否存在差异,提高医务工作者,特别是呼吸科医生对该病的认知、诊断及治疗水平。方法收集于2011年1月至2016年7月期间就诊于郑州大学第一附属医院的所有诊断为复发性多软骨炎患者的临床资料,具体包括患者性别、年龄、临床表现、首诊情况、实验室检查如白细胞(white blood cell,WBC)、血红蛋白(Hemoglobinemia,HB)、血小板(blood platelet,PLT)、红细胞沉降率(erythrocyte sedimentation rate,ESR)、C-反应蛋白(C-reactive protein,CRP)、D-二聚体(D-Dimer,D-D)、纤维蛋白原(fibrinogen,FIB)、类风湿因子(rheumatoid factor,RF)、影像学表现、其他检查(气管镜、肺功能)、组织病理、合并症、治疗情况等。并对总样本进行分组,累及呼吸道组和未累及呼吸道组,分别对两组的性别、平均发病年龄、城乡分布、误诊率、血清学指标、确诊时间、合并症、转归情况进行比较。运用SPSS 21.0软件进行统计绘图及统计学分析。对其符合正态分布的计量变量资料以`X±s表示,两组独立样本之间比较采用t检验,四格表计量变量资料采用χ2检验,单自变量危险因素分析采用Logistic回归,检验水准均α=0.05。结果总样本结果:1.临床特点:(1)性别、年龄:48例患者中,男30例,女18例,发病年龄11~72岁,平均发病年龄(44±14)岁。(2)累及部位:累及呼吸道者占首位,共23例,其次分别是耳部累及者18例,关节累及者17例,眼部累及者12例,鼻部累及者8例,皮肤累及者3例。(3)呼吸道症状表现:23例呼吸道受累患者症状,咳嗽18例、咳痰17例、胸闷14例、呼吸困难6例、胸痛5例、声音嘶哑4例、气喘3例、饮水呛咳1例。2.诊断情况:确诊为RP者16例,漏诊或误诊者32例,误诊为呼吸系统疾病者21例,耳部疾病者3例,眼部疾病者1例,皮肤疾病者1例,自身免疫性疾病者1例,发热待查者5例。3.实验室检查:48例RF中,WBC升高37例、HB下降30例、PLT升高21例、ESR升高27例、CRP升高31例、FIB升高25例、D-D升高25例、RF升高14例。4.影像学检查:23例行胸部CT;2例行胸部MRI。5.肺功能:10例行肺功能。6.气管镜:10例患者行支气管镜检查。7.组织病理:6例行组织活检。8.合并症:15例同时合并其他疾病。9.治疗情况:9例给予对症治疗,未应用糖皮质激素;37例均应用糖皮质激素治;2例应用生物制剂;48例RP患者中,8例死亡。呼吸道受累组和呼吸道未受累组比较结果:1.呼吸道受累的RP患者与呼吸道未受累的RP患者相比,两组之间在性别、年龄、城乡分布、确诊时间及血清学中部分指标如WBC、HB、PLT、RF方面无差异。2.两组之间在误诊率、合并症、转归情况及血清学中部分指标如ESR、CRP、D-D、FIB方面存在差异。结论复发性多软骨炎可单器官受累,亦可侵多个器官或组织,约半数患者累及呼吸道。累及呼吸道的RP患者比未累及呼吸道的RP患者误诊率高、合并症发生率高、治疗效果差,累及呼吸道者症状偏重且病死率高,因此能够做到早期诊断、及时治疗,对于提高患者生存期、提高患者生活质量具有重要意义。
[Abstract]:The background of relapsing polychondritis (relapsing polychondritis RP) is a kind of degenerative cartilage recurrent inflammation characterized by a relatively rare disease, its etiology and mechanism is not clear, it has close relationship with the immune response to the body. The body is affected by external stimuli such as inflammation, trauma or influencing factors allergies, effects on cartilage matrix, which exposed the body with the same antigen structure of cartilage matrix components on cartilage tissue or cause (such as eye conjunctiva, cornea, sclera, choroid and optic nerve intima, vitreous body; basement membrane; tracheal submucosal synovial; heart valve; aortic medial and the inner layer of connective tissue; glomerular and tubular basement membrane) damage. A single organ or tissue lesions, also involving multiple organs or tissues, often involving organs or tissues have eyes, ears, nose, larynx, trachea And bronchi, joints, heart and kidney. About half of the patients in the respiratory system, or symptoms or arising in the course, the progression of chronic, recurrent, easily misdiagnosed or missed diagnosis. The disease incidence rate is low, the pathogenesis is not clear, atypical clinical manifestations, disease characteristics are not prominent in addition, the lack of specific serological indexes, imaging examination is not easy to be found, the cartilage biopsy is the gold standard for the diagnosis of the disease, but for biopsy is not easy to obtain, for traumatic operation, and a series of factors on the disease literature is less, resulting in the clinical medical workers to the lack of awareness of disease the above reasons to increase the difficulty of diagnosis the disease, prolong hospitalization time, increase the rate of misdiagnosis or missed diagnosis rate, increase the economic burden on patients and society. Therefore, to understand the disease epidemic characteristics, pay attention to the disease Pro The clinical manifestation, pathogenesis research of the disease, laboratory index to study its specificity, improve the understanding of characteristics of the disease image, familiar with the diagnostic criteria of the disease, to explore effective treatment, strengthen the follow-up work with the prognosis of patients is imperative. Objective to investigate the epidemiological characteristics of relapsing polychondritis with clinical manifestations. Laboratory examination and imaging examination, other specific (pulmonary function, bronchoscopy), complications, therapeutic methods and curative effect. To investigate the respiratory tract involvement group and respiratory unaffected groups in gender, age of onset, initial misdiagnosis rate, serological indexes, diagnosis time, whether there are differences in complications and prognosis, improve medical staff in particular, the Department of respiration doctor cognition of the disease, the diagnosis and treatment level. All diagnostic methods collected from January 2011 to July 2016 during treatment from the First Affiliated Hospital of Zhengzhou University for The clinical data of patients with recurrent polychondritis, including gender, age, clinical manifestation, laboratory examination of first diagnosis, such as white blood cells (white blood cell, WBC), hemoglobin (Hemoglobinemia, HB), platelet (blood platelet, PLT), erythrocyte sedimentation rate (erythrocyte sedimentation, rate, ESR) C-, C-reactive protein (C-reactive protein, CRP), two D- dimer (D-Dimer, D-D), fibrinogen (fibrinogen, FIB), rheumatoid factor (rheumatoid, factor, RF), imaging findings, other tests (bronchoscopy, lung function), pathology, complications, treatment and so on. For grouping samples, involving the respiratory group and not involving the respiratory group, respectively on two groups of gender, the average age of onset, distribution of urban and rural areas, the rate of misdiagnosis, serological indexes, diagnosis time, complications, prognosis were compared. Statistical graphics and statistics by using SPSS 21 software 鏋，

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