本文關鍵詞：48例復發(fā)性多軟骨炎臨床特點及療效分析　出處：《鄭州大學》2017年碩士論文　論文類型：學位論文

  更多相關文章： 復發(fā)性多軟骨炎 累及呼吸道 誤診率 療效

【摘要】：背景復發(fā)性多軟骨炎(relapsing polychondritis,RP)是一種以軟骨組織復發(fā)性退化性炎癥為特點的較為少見的疾病,其具體發(fā)病原因及機制尚不清楚,目前認為與機體的自身免疫反應有密切關系。機體受外界刺激如炎癥、外傷或過敏等因素的影響,作用于軟骨基質,使其暴露出抗原,致使機體對軟骨組織或具有相同軟骨基質成分的組織結構(如眼部的結膜、角膜、鞏膜、葡萄膜、視神經內膜、玻璃體;氣管粘膜下的基底膜;關節(jié)滑膜;心臟瓣膜;主動脈中層和內層結締組織;腎小球及腎小管基底膜等)造成破壞�？蓡蝹�器官或組織病變,亦可累及多個器官或組織,常累及的器官或組織有眼、耳、鼻、喉、氣管及支氣管、關節(jié)、心臟瓣膜和腎等。約半數(shù)患者可累及呼吸系統(tǒng),或以首發(fā)癥狀或是病程中出現(xiàn),慢性進展,反復發(fā)作,易被誤診或漏診。該病發(fā)病率低,發(fā)病機制尚不明確,臨床表現(xiàn)不典型,病變特征不突出,加之缺乏特異性的血清學指標,影像學檢查不易被發(fā)現(xiàn),其軟骨組織病理活檢是診斷該病的金標準,但因獲取活檢組織不易獲得,需創(chuàng)傷性操作,且關于該病的文獻較少等一系列因素,導致臨床醫(yī)務工作者對該病認識上的缺乏,以上種種原因給確診此病增加了難度,進而延長患者的住院時間、增大誤診率或漏診率,增加患者及社會的經濟負擔。因此,了解該病的流行病學特征、重視該病的臨床表現(xiàn)、深入探究該病的發(fā)病機制、研究其特異性的實驗室指標、提高對該病影像學特點的認識、熟悉該病的診斷標準、探究行之有效的治療方案、加強患者預后的隨訪工作勢在必行。目的探討復發(fā)性多軟骨炎的流行病學特征、臨床表現(xiàn)、實驗室及影像學檢查、其他特異性檢查(肺功能、氣管鏡)、合并癥、治療方法及療效。探討呼吸道受累組和呼吸道未受累組在性別、發(fā)病年齡、首次誤診率、血清學指標、確診時間、合并癥及轉歸情況是否存在差異,提高醫(yī)務工作者,特別是呼吸科醫(yī)生對該病的認知、診斷及治療水平。方法收集于2011年1月至2016年7月期間就診于鄭州大學第一附屬醫(yī)院的所有診斷為復發(fā)性多軟骨炎患者的臨床資料,具體包括患者性別、年齡、臨床表現(xiàn)、首診情況、實驗室檢查如白細胞(white blood cell,WBC)、血紅蛋白(Hemoglobinemia,HB)、血小板(blood platelet,PLT)、紅細胞沉降率(erythrocyte sedimentation rate,ESR)、C-反應蛋白(C-reactive protein,CRP)、D-二聚體(D-Dimer,D-D)、纖維蛋白原(fibrinogen,FIB)、類風濕因子(rheumatoid factor,RF)、影像學表現(xiàn)、其他檢查(氣管鏡、肺功能)、組織病理、合并癥、治療情況等。并對總樣本進行分組,累及呼吸道組和未累及呼吸道組,分別對兩組的性別、平均發(fā)病年齡、城鄉(xiāng)分布、誤診率、血清學指標、確診時間、合并癥、轉歸情況進行比較。運用SPSS 21.0軟件進行統(tǒng)計繪圖及統(tǒng)計學分析。對其符合正態(tài)分布的計量變量資料以`X±s表示,兩組獨立樣本之間比較采用t檢驗,四格表計量變量資料采用χ2檢驗,單自變量危險因素分析采用Logistic回歸,檢驗水準均α=0.05。結果總樣本結果:1.臨床特點:(1)性別、年齡:48例患者中,男30例,女18例,發(fā)病年齡11~72歲,平均發(fā)病年齡(44±14)歲。(2)累及部位:累及呼吸道者占首位,共23例,其次分別是耳部累及者18例,關節(jié)累及者17例,眼部累及者12例,鼻部累及者8例,皮膚累及者3例。(3)呼吸道癥狀表現(xiàn):23例呼吸道受累患者癥狀,咳嗽18例、咳痰17例、胸悶14例、呼吸困難6例、胸痛5例、聲音嘶啞4例、氣喘3例、飲水嗆咳1例。2.診斷情況:確診為RP者16例,漏診或誤診者32例,誤診為呼吸系統(tǒng)疾病者21例,耳部疾病者3例,眼部疾病者1例,皮膚疾病者1例,自身免疫性疾病者1例,發(fā)熱待查者5例。3.實驗室檢查: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.影像學檢查:23例行胸部CT;2例行胸部MRI。5.肺功能:10例行肺功能。6.氣管鏡:10例患者行支氣管鏡檢查。7.組織病理:6例行組織活檢。8.合并癥:15例同時合并其他疾病。9.治療情況:9例給予對癥治療,未應用糖皮質激素;37例均應用糖皮質激素治;2例應用生物制劑;48例RP患者中,8例死亡。呼吸道受累組和呼吸道未受累組比較結果:1.呼吸道受累的RP患者與呼吸道未受累的RP患者相比,兩組之間在性別、年齡、城鄉(xiāng)分布、確診時間及血清學中部分指標如WBC、HB、PLT、RF方面無差異。2.兩組之間在誤診率、合并癥、轉歸情況及血清學中部分指標如ESR、CRP、D-D、FIB方面存在差異。結論復發(fā)性多軟骨炎可單器官受累,亦可侵多個器官或組織,約半數(shù)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患者誤診率高、合并癥發(fā)生率高、治療效果差,累及呼吸道者癥狀偏重且病死率高,因此能夠做到早期診斷、及時治療,對于提高患者生存期、提高患者生活質量具有重要意義。
[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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