本文選題：炎性肌病 + 肺間質(zhì)病變　； 參考：《北京協(xié)和醫(yī)學院》2016年博士論文

【摘要】：目的：探索與炎性肌病(Inflammatory myopathy, IM)合并肺間質(zhì)病變(interstitial lung disease, ILD)相關(guān)的臨床危險因素。方法：對北京協(xié)和醫(yī)院2012年5月至2016年4月納入中國風濕病數(shù)據(jù)中心(ChineseRheumatism Data Center, CRDC) IM隨訪隊列的患者進行橫斷面分析。比較皮肌炎(dermatomyositis, DM)和多發(fā)性肌炎(polymyositis, PM)合并ILD(IM-ILD組)和未合并ILD (IM-non-ILD組)患者的臨床表現(xiàn)、實驗室檢查、輔助檢查以及活動性和損傷評分。采用多因素Logistic回歸分析確定與ILD相關(guān)的獨立危險因素。結(jié)果：共218例IM患者納入北京協(xié)和醫(yī)院的CRDC隨訪隊列。其中ILD組105例(48.2%)。ILD組與non-ILD組患者的年齡(48.8±12.4歲vs.45.6±15.3歲,P=0.089)、性別(女性比例,68.6%vs.76.1%,P=0.213)、IM病程(21.4±29.3月vs.22.4±29.8月,P=0.798)和疾病類型(DM比例,85.7%vs.77.9%,P=0.135)均未見顯著性差異。多因素Logistic回歸表明抗Jo-1抗體陽性(OR=9.146,95%CI 1.614-51.818,P=0.012)、皮膚紅斑(OR=6.326,95%CI 2.528-15.831,P0.001)、技工手(OR=3.311,95%CI 1.450-7.559,P=0.004)、皮膚破潰(OR=3.277,95%CI 1.235-8.696,P=0.017)、關(guān)節(jié)炎(OR=2.830,95%CI 1.265-6.333,P=0.011)、皮疹分布(OR=2.130,95%CI1.407-2.882,P0.001)、既往最重功能狀態(tài)分級(OR=1.695,95%CI 1.189-2.415,P=0.004)具有統(tǒng)計學差異。結(jié)論：在IM患者伴抗Jo-1抗體陽性、廣泛特征性皮疹、關(guān)節(jié)炎及既往最重功能狀態(tài)分級高時,應注意篩查潛在的ILD,并需要緊密隨訪ILD的發(fā)生。目的：探究與炎性肌病(inflammatory myositis, IM)合并肺間質(zhì)病變(interstitial lung disease, ILD)相關(guān)的血清標志物及其臨床意義。方法：檢測北京協(xié)和醫(yī)院納入中國風濕病數(shù)據(jù)中心(Chinese Rheumatism Data Center, CRDC)IM隊列的患者血清中Krebs von den Lungen 6(KL-6)、肺表面活性蛋白A(SP-A)、肺表面活性蛋白D(SP-D)、幾丁質(zhì)酶3樣蛋白1(YKL-40/CH3L1)、卵泡抑素樣蛋白1 (FSTL-1)、金屬蛋白酶7(MMP-7)的表達水平,比較IM合并ILD者(IM-ILD組)、未合并ILD者(IM-nonILD組)及健康對照人群(HC組)間上述血清標記物的表達差異,及其與IM其他臨床特征的相關(guān)性。根據(jù)血清標志物水平診斷肺間質(zhì)病變的敏感性和特異性繪制ROC曲線,確定cut-off值。結(jié)果：共納入IM患者149例,其中合并ILD者71例(47.7%%)。IM-ILD組KL-6水平較IM-nonILD組升高(1g KL-6,2.88±0.38 vs 2.51±0.31,P0.001)而IM患者兩組血清FSTL-1則顯著低于健康對照組(9.07±6.84,11.04±13.83 vs 23.47±9.40,P=0.001)。以ROC曲線確定KL-6水平461.5 U/mI作為診斷ILD的cut-off值,則其診斷敏感性為73.2%,特異性為78.2%。血清SP-A、SP-D、YKL-40、MMP-7血清水平在IM-ILD、IM-nonILD及健康對照組間差異均無統(tǒng)計學顯著性。結(jié)論：KL-6可以作為預測IM患者合并ILD的血清學指標。SP-A、SP-D、YKL-40、FSTL-1及MMP-7與IM-ILD的相關(guān)性及其在ILD發(fā)生中的機制尚有待進一步研究。目的：探究炎性肌病(Inflammatory myopathies, IM)并發(fā)惡性腫瘤的患病率、臨床特點和可能的危險因素。方法：收集2008年-2014年在北京協(xié)和醫(yī)院診斷為多發(fā)性肌炎(Polymyositis, PM)或皮肌炎(Dermatomyositis, DM)并發(fā)惡性腫瘤的的患者住院資料(腫瘤組),并以未合并惡性腫瘤的IM患者按1：2進行性別和年齡匹配(對照組)。比較IM并發(fā)腫瘤組及對照組間的臨床和實驗室檢查特點的差異。采用多因素Logistic回歸確定與IM并發(fā)惡性腫瘤相關(guān)的獨立危險因素。結(jié)果：共納入IM患者753例。并發(fā)惡性腫瘤者40例(5.3%),其中男性14例,女性26例。PM 7例,DM 32例,1例伴抗合成酶綜合征(Anti-synthetase syndrome,ASS)。腫瘤組患者入院平均年齡為56.2±13.4歲。14例(35%)患者腫瘤診斷時間早于IM(42.10±59.35月),8例(20%)患者同時診斷,18例(45%)患者腫瘤的診斷時間晚于IM(19.10±35.03月)。腫瘤好發(fā)部位前4位依次為：乳腺癌(n=9,17.5%)、卵巢癌(n=7,15%)、肺癌(n=5,12.5%)和鼻咽癌(n=4,10.0%)。多因素Logistic回歸顯示胃腸道受累(包括出血、穿孔、潰瘍、梗阻等)是IM并發(fā)腫瘤的獨立危險因素(OR=4.604,95%CI 1.146-18.501,P=0.031),而肺間質(zhì)病變則是其保護性因素(OR=0.150,95%C10.049-0.455,P=0.001)。結(jié)論：部分IM患者可并發(fā)惡性腫瘤。對于出現(xiàn)胃腸道受累且未合并肺間質(zhì)病變的患者應積極篩查腫瘤。
[Abstract]:Objective: To explore the clinical risk factors associated with Inflammatory myopathy (IM) combined with interstitial lung disease (ILD). Methods: transection of patients in Peking Union Medical College Hospital from May 2012 to April 2016, which were included in the follow-up cohort of the Chinese rheumatism data center (ChineseRheumatism Data Center, CRDC) IM. Surface analysis. Comparison of clinical manifestations, laboratory tests, auxiliary examinations, activity and injury scores in patients with dermatomyositis (DM) and polymyositis (polymyositis, PM) combined with ILD (group IM-ILD) and unincorporated ILD (IM-non-ILD group). Multiple factor Logistic regression analysis was used to determine independent risk factors associated with ILD. A total of 218 patients with IM were included in the CRDC follow-up cohort in Peking Union Medical College Hospital, of which 105 (48.2%) group.ILD and non-ILD group were (48.8 + 12.4, vs.45.6 + 15.3, P=0.089), sex (female ratio, 68.6%vs.76.1%, P=0.213), IM course (21.4 + 29.3 months vs.22.4 + 29.8 months, P=0.798) and disease types. No significant difference was found. Multiple factor Logistic regression showed that anti Jo-1 antibody positive (OR=9.146,95%CI 1.614-51.818, P=0.012), skin erythema (OR=6.326,95%CI 2.528-15.831, P0.001), technician hands (OR=3.311,95%CI 1.450-7.559, P=0.004), skin rupture (OR=), arthritis OR=2.130,95%CI1.407-2.882 (P0.001), OR=1.695,95%CI 1.189-2.415, P=0.004 (P=0.004) have statistical differences. Conclusion: in IM patients with anti Jo-1 antibody positive, extensive characteristic rash, arthritis, and previous most severe functional status classification, attention should be paid to screening potential ILD and need to be closely followed. Visit ILD. Objective: To explore the serum markers associated with inflammatory myositis (IM) combined with interstitial lung disease (ILD) and its clinical significance. Methods: Peking Union Medical College Hospital was included in the patients' serum of the Chinese rheumatic data center (Chinese Rheumatism Data Center). Krebs von den Lungen 6 (KL-6), lung surface active protein A (SP-A), lung surface active protein D (SP-D), chitinase 3 like protein 1 (YKL-40/CH3L1), follicle statin like protein 1 (FSTL-1), and metalloproteinase 7 (MMP-7) The difference in the expression of the clear markers and the correlation with other clinical features of IM. According to the sensitivity and specificity of the diagnosis of pulmonary interstitial lesions by the serum markers level, the ROC curve was plotted and the cut-off value was determined. Results: a total of 149 patients with IM were included, of which 71 (47.7%%).IM-ILD group had a higher KL-6 level than the IM-nonILD group (1g KL-6,2.88 + 0.38). Vs was 2.51 + 0.31, P0.001), while the serum FSTL-1 in two groups of IM patients was significantly lower than that in the healthy control group (9.07 + 6.84,11.04 + 13.83 vs 23.47 + 9.40, P=0.001). The KL-6 level 461.5 U/mI was determined as the cut-off value of the ILD, and the sensitivity was 73.2%. The difference between onILD and the healthy control group was not statistically significant. Conclusion: KL-6 can be used as a serological index for predicting ILD in IM patients.SP-A, the correlation between SP-D, YKL-40, FSTL-1 and MMP-7 and IM-ILD and the mechanism in the occurrence of ILD are still to be further studied. The prevalence, clinical characteristics, and possible risk factors of tumor. Methods: data of hospitalized patients (tumor groups) diagnosed with Polymyositis (PM) or dermatomyositis (Dermatomyositis, DM) in Peking Union Medical College Hospital in -2014 2008 were collected, and IM patients who were not combined with malignant tumors were given sex and sex according to 1:2. Age matching (control group). Compare the differences in clinical and laboratory characteristics between IM and control groups. Multiple factor Logistic regression was used to determine independent risk factors associated with IM complicated with malignant tumors. Results: a total of 753 cases of IM patients were included. 40 cases of malignant tumors (5.3%), including 14 males, 26.PM 7 cases, DM 3. 2 cases, 1 cases with Anti-synthetase syndrome (ASS). The average age of the tumor group was 56.2 + 13.4 years old (35%), the diagnosis time of the tumor was earlier than IM (42.10 + 59.35 months), 8 (20%) patients were simultaneously diagnosed, 18 (45%) patients were later than IM (19.10 + 35.03 months). Breast cancer (n=9,17.5%), ovarian cancer (n=7,15%), lung cancer (n=5,12.5%) and nasopharyngeal carcinoma (n=4,10.0%). Multifactorial Logistic regression shows that gastrointestinal involvement (including bleeding, perforation, ulcers, obstruction, etc.) is an independent risk factor for IM complicated with tumors (OR=4.604,95%CI 1.146-18.501, P=0.031), and interstitial lung disease is a protective factor (OR=0.150,95%C1). 0.049-0.455, P=0.001) conclusion: some IM patients may be associated with malignant tumors. Patients with gastrointestinal involvement and no pulmonary interstitial lesions should be actively screened for cancer.
【學位授予單位】：北京協(xié)和醫(yī)學院
【學位級別】：博士
【學位授予年份】：2016
【分類號】：R563;R593.2

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