幼年皮肌炎94例臨床分析及影響幼年皮肌炎預(yù)后的危險(xiǎn)因素分析
發(fā)布時(shí)間：2018-09-14 15:10
【摘要】：背景和目的幼年皮肌炎是一種少見的風(fēng)濕免疫性疾病,也是兒童時(shí)期最常見的炎性肌病,其以對(duì)稱性的近端肌無力和特殊皮疹為特點(diǎn),其他系統(tǒng)亦可受累。通過分析94例幼年皮肌炎患兒的病例特點(diǎn)及隨訪情況,總結(jié)幼年皮肌炎患兒的臨床特點(diǎn),提出影響幼年皮肌炎預(yù)后的可能因素。方法選取1990年至2016年在北京協(xié)和醫(yī)院住院診治的幼年皮肌炎患兒,回顧性分析患兒的人口學(xué)特征、臨床表現(xiàn)、輔助檢查結(jié)果、治療情況、隨診情況及病程中出現(xiàn)的并發(fā)癥。根據(jù)隨診情況將患兒分為單病程組與預(yù)后不良組,通過統(tǒng)計(jì)學(xué)方法分析,得出影響幼年皮肌炎患兒預(yù)后的可能危險(xiǎn)因素。結(jié)果本研究共納入符合診斷標(biāo)準(zhǔn)的病例94例,其中經(jīng)典型皮肌炎90例,臨床無肌病皮肌炎4例,其中2例病例因合并肺間質(zhì)病變、肺部感染死亡。堅(jiān)持隨訪3年以上病例44例,最長(zhǎng)隨訪時(shí)間194個(gè)月,31人達(dá)到臨床緩解,臨床緩解率為70.45%,達(dá)到臨床緩解的平均時(shí)間為7.00(4.50-17.00)個(gè)月,其中15人(34.09%)臨床治愈,達(dá)到臨床治愈的平均時(shí)間為50.46個(gè)月;8人(18.18%)達(dá)到臨床緩解,但口服藥物至少有一種尚未減停;8人(18.18%)在藥物減量過程中曾出現(xiàn)疾病復(fù)發(fā),但末次復(fù)發(fā)后可滿足臨床緩解標(biāo)準(zhǔn);12人(27.27%)病程遷延,始終無法達(dá)到臨床緩解;1例(2.27%)激素減量過程中合并EBV感染,繼發(fā)噬血細(xì)胞綜合征。將病情遷延、病情反復(fù)、死亡病例納入預(yù)后不良組,與單病程組進(jìn)行比較,預(yù)后不良組平均起病年齡6.05±3.21歲,單一病程組平均起病年齡6.77±3.40歲,但無統(tǒng)計(jì)學(xué)差異;預(yù)后不良組確診至起病平均時(shí)間5.5個(gè)月,單病程組4個(gè)月,無統(tǒng)計(jì)學(xué)差異;存在呼吸系統(tǒng)受累共10例,其中預(yù)后不良組8例,單病程組2例,有統(tǒng)計(jì)學(xué)差異(p0.05);病程中出現(xiàn)皮下鈣化共16例,其中預(yù)后不良組11例,單病程組5例,有統(tǒng)計(jì)學(xué)差異(p0.05)。將起病年齡、未治療時(shí)間、是否出現(xiàn)皮下鈣化、是否存在呼吸系統(tǒng)受累共同進(jìn)行Logistic回歸分析,其中存在呼吸系統(tǒng)受累兩組間存在顯著差異(p0.05)。結(jié)論幼年皮肌炎可呈現(xiàn)多系統(tǒng)損害,多數(shù)病例預(yù)后良好,70%以上病例可達(dá)到臨床緩解,呼吸系統(tǒng)受累是幼年皮肌炎預(yù)后不良的危險(xiǎn)因素,幼年皮肌炎患者無論有無呼吸系統(tǒng)臨床癥狀均應(yīng)完善呼吸系統(tǒng)評(píng)估。
[Abstract]:Background and objective infantile dermatomyositis is a rare rheumatic immune disease and the most common inflammatory myopathy in childhood. It is characterized by symmetrical proximal myasthenia and special rash. Other systems may also be involved. The clinical features of 94 cases of infantile dermatomyositis and their follow-up were analyzed, and the possible factors influencing the prognosis of juvenile dermatomyositis were put forward. Methods Children with infantile dermatomyositis, who were hospitalized in Peking Union Hospital from 1990 to 2016, were retrospectively analyzed for their demographic characteristics, clinical manifestations, auxiliary examination results, treatment, follow-up and complications in the course of disease. The children were divided into single course group and poor prognosis group according to the follow up. The possible risk factors affecting the prognosis of infant dermatomyositis were obtained by statistical analysis. Results 94 cases were included in this study, including 90 cases of classic dermatomyositis and 4 cases of clinical non-myopathy dermatomyositis. 2 cases died of pulmonary infection due to pulmonary interstitial disease. 44 cases were followed up for more than 3 years. The longest follow-up time was 194 months. The clinical remission rate was 70.45, and the average time to clinical remission was 7.00 (4.50-17.00) months. Among them, 15 patients (34.09%) were cured clinically. The average time to achieve clinical cure was 50.46 months or 8 (18.18%), but at least one oral drug had not been reduced or stopped. 8 (18.18%) had relapsed in the course of drug reduction. However, 12 patients (27.27%) could meet the criteria of clinical remission after the last relapse, and the course of disease was delayed, and one case (2.27%) with EBV infection and secondary hemophagocytic syndrome was unable to achieve clinical remission. The average onset age of poor prognosis group was 6.05 鹵3.21 years old, and the average onset age of single course group was 6.77 鹵3.40 years, but there was no statistical difference. The mean time from diagnosis to onset was 5.5 months in poor prognosis group and 4 months in single course group, and there were 10 cases of respiratory system involvement, including 8 cases in poor prognosis group and 2 cases in single course group. There were 16 cases of subcutaneous calcification in the course of the disease, including 11 cases of poor prognosis group and 5 cases of single course group (p0.05). The onset age, untreated time, subcutaneous calcification and respiratory system involvement were analyzed by Logistic regression analysis. There was significant difference between the two groups (p0.05). Conclusion Young dermatomyositis may present multiple system damage, and the prognosis of most of the cases can reach clinical remission in more than 70% cases. Respiratory system involvement is the risk factor of poor prognosis of juvenile dermatomyositis. Patients with juvenile dermatomyositis should improve the assessment of respiratory system regardless of the clinical symptoms of respiratory system.
【學(xué)位授予單位】：北京協(xié)和醫(yī)學(xué)院
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2017
【分類號(hào)】：R725.9
本文編號(hào)：2243070
[Abstract]:Background and objective infantile dermatomyositis is a rare rheumatic immune disease and the most common inflammatory myopathy in childhood. It is characterized by symmetrical proximal myasthenia and special rash. Other systems may also be involved. The clinical features of 94 cases of infantile dermatomyositis and their follow-up were analyzed, and the possible factors influencing the prognosis of juvenile dermatomyositis were put forward. Methods Children with infantile dermatomyositis, who were hospitalized in Peking Union Hospital from 1990 to 2016, were retrospectively analyzed for their demographic characteristics, clinical manifestations, auxiliary examination results, treatment, follow-up and complications in the course of disease. The children were divided into single course group and poor prognosis group according to the follow up. The possible risk factors affecting the prognosis of infant dermatomyositis were obtained by statistical analysis. Results 94 cases were included in this study, including 90 cases of classic dermatomyositis and 4 cases of clinical non-myopathy dermatomyositis. 2 cases died of pulmonary infection due to pulmonary interstitial disease. 44 cases were followed up for more than 3 years. The longest follow-up time was 194 months. The clinical remission rate was 70.45, and the average time to clinical remission was 7.00 (4.50-17.00) months. Among them, 15 patients (34.09%) were cured clinically. The average time to achieve clinical cure was 50.46 months or 8 (18.18%), but at least one oral drug had not been reduced or stopped. 8 (18.18%) had relapsed in the course of drug reduction. However, 12 patients (27.27%) could meet the criteria of clinical remission after the last relapse, and the course of disease was delayed, and one case (2.27%) with EBV infection and secondary hemophagocytic syndrome was unable to achieve clinical remission. The average onset age of poor prognosis group was 6.05 鹵3.21 years old, and the average onset age of single course group was 6.77 鹵3.40 years, but there was no statistical difference. The mean time from diagnosis to onset was 5.5 months in poor prognosis group and 4 months in single course group, and there were 10 cases of respiratory system involvement, including 8 cases in poor prognosis group and 2 cases in single course group. There were 16 cases of subcutaneous calcification in the course of the disease, including 11 cases of poor prognosis group and 5 cases of single course group (p0.05). The onset age, untreated time, subcutaneous calcification and respiratory system involvement were analyzed by Logistic regression analysis. There was significant difference between the two groups (p0.05). Conclusion Young dermatomyositis may present multiple system damage, and the prognosis of most of the cases can reach clinical remission in more than 70% cases. Respiratory system involvement is the risk factor of poor prognosis of juvenile dermatomyositis. Patients with juvenile dermatomyositis should improve the assessment of respiratory system regardless of the clinical symptoms of respiratory system.
【學(xué)位授予單位】：北京協(xié)和醫(yī)學(xué)院
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2017
【分類號(hào)】：R725.9
【參考文獻(xiàn)】
相關(guān)期刊論文 前1條
1 舒曉明;王國(guó)春;;炎性肌病的臨床評(píng)估工具介紹[J];中華風(fēng)濕病學(xué)雜志;2011年07期
，本文編號(hào)：2243070
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