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纸质出版:2010
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骆文静,蒲传强,石强.90例多发性肌炎和皮肌炎预后影响因素随访研究[J].陆军军医大学学报(原第三军医大学学报),2010,32(08):842-845.
Luo Wenjing,Pu Chuanqiang,Shi Qiang.Factors influencing prognosis of 90 polymyositis and dermatomyositis patients[J].J Amry Med Univ (J Third Mil Med Univ),2010,32(08):842-845.
目的 研究多发性肌炎(polymyositis,PM)和皮肌炎(dermatomyositis,DM)的预后及其影响因素。 方法 根据Bohan和Peter诊断标准,选择2000-2008年本院DM(n=60)和PM(n=30)住院患者作为研究对象。患者随访至2009年8月或死亡时。性别、发病年龄、病程、血清肌酸激酶、肺病、心脏受累、伴发结缔组织病、恶性肿瘤、强的松、丙种球蛋白及免疫抑制剂治疗作为评估预后的指标。分析20例行肌活检检查的特点。 结果 90例患者平均51岁(6~74岁),其中男性29例,女性61例。四肢近端肌肉为最常见受累肌(83.3%),其次依次为颈肌(25.6%)、咽喉肌(12.2%)和咀嚼肌(2.2%)。42例(46.7%)并发肺部疾患,其中29例(32.2%)确诊为肺间质病变。13例(14.4%)合并心脏受累。13例合并其他结缔组织病,其中DM合并结缔组织病9例,PM合并4例。PM和DM各合并恶性肿瘤2例。20例行肌活检示11例有典型炎性细胞浸润,4例有皮肌炎病理学改变,未见明显异常2例。随访期间死亡18例,其中PM死亡5例(16.67%),DM死亡13例(21.67%)。发病1年生存率为90.0%,5年生存率为84.4%,9年生存率为80.0%。痊愈率为22.2%,部分缓解率36.7%,复发率为20.0%。发病年龄较大(P=0.003 8)、合并间质性肺炎(P=0.013 3)和恶性肿瘤(P=0.004 9)是主要死因。恶性肿瘤(RR=6.34,P=0.001 2)是预后差的预测因素,持续激素治疗(RR=0.05
P=0.004 7)和持续免疫抑制治疗(RR=0.10,P=0.027 6)则是PM和DM的保护因素。 结论 58.9%的患者可获得痊愈或部分缓解。发病年龄较大,合并间质性肺炎、恶性肿瘤是PM和DM预后差主要因素,而持续激素治疗及免疫抑制剂治疗则是保护因素。
Objective To study the prognosis of polymyositis (PM) and dermatomyositis (DM) patients and its influence factors. Methods Sixty DM and 30 PM patients diagnosed according to the diagnostic criteria of Bohan and Peter in our hospital during 2000-2008 were used as study subjects. The patients were followed up till their death or to August 2009. Gender
age of disease onset
disease course
serum creatine kinase
interstitial lung disease
heart involvement
connective tissue disease (CTD)
malignancies
and treatment with corticosteroids
immunoglobulin and immunosuppressive agents were assessed as predictive factors for the prognosis of patients. Characteristics of muscular biopsy from 20 cases were analyzed. Results The median age of the 90 patients (29 males and 61 females) was 51 years (range 6-74 years). The male and female ratio was 1∶2. The most commonly involved muscles were the proximal muscles of limbs (83.3%)
followed by neck muscles (25.6%)
laryngea pharyngeal muscles (12.2%) and masticatory muscles (2.2%). Among the 42 patients(46.7%)with lung disease
interstitial lung disease and hear involvement were found in 29 (32.2%)and 13(14.4%)patients
respectively. Of the 13 patients complicated by connective tissue disease
DM and PM accompanying connective tissue disease were diagnosed in 9 and 4
respectively
and DM and PM accompanying malignancies were observed in 2 patients. Muscular or skin biopsy was performed for 23 patients
which showed typical inflammatory infiltration in 13
dermatomyositis in 3
and no significant lesion in 2 patients
respectively. Of the 18 patients who died during the follow-up
5 (16.67%) and 13 (21.67%) died of PM and DM
respectively. Seventy-two patients survived. Their 1-
5-
and 9- year survival rate was 90%
84.4%
and 80%
respectively. The complete and partial remission rate was 22.2% and 36.7%
respectively
with a relapse rate of 20%. Advanced age of disease onset (P=0.003 8)
interstitial lung disease (P=0.011 3) and malignancies (P=0.004 9) were main causes of death. Malignancy(RR=6.34,P=0.001 2)was the factor for poor prognosis and long-term treatment with hormones and immunosuppressive agents is the protective factor for PM and DM. Conclusion Complete and partial remission can be achieved in 58.9% patients with DM and PM. Advanced age of disease onset
interstitial lung disease and malignancy are the factors for poor prognosis of such patients. Long-term treatment with corticosteroids and immunosuppressive agents are the protective factors.
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