The Prognosis And Its Prognosis Factors Of Aadult Classic Dermatomyositis And Clinical Amyopathic Dermatomyositis

Background:By now,there are relatively rare researches from large sample about the long prognosis of Aadult Dermatomyositis(DM) and Clinical Amyopathic Dermatomyositis(CADM) due to the disease is raletively rare, and the results are various because the diference on sample size and other study factors.Objectives:1.To investigate the long prognosis of DM/CADM patients and analyse the risk factors of its prognosis,and give a clue for diagnosis and treatment.2.To investigate the clinical characteristic and the prognosis of interstitial lung disease(ILD) complicated with DM/CADM.Methods:Part 1 545 cases of DM and 153 cases of CADM admitted to our hospital between 1993 and 2009 were reviewed.Collecting patients'individual and clinical information from medical history,and all patients were surveyed using the same standard questionnaire including current survival conditions(survival and death),the death time and the disease-related death and complications and so on.To get the survival rate and survival curve by Kaplan-Meier survival estimate.To get the risk factors of progress by Log-rank test and Cox regression.It is considered significant difference when p=0.05.Part 2 To review the clinical manifestation and high resolution computed tomography(HRCT) manifestation and prognosis of 122 patients complicated with ILD from the 698 patients above.All patients were divided into two groups:one was complicated with acute ILD and the other was complicated chronic ILD.The clinical manifestation and the prognosis were compared between the two groups,and the survival curve were drawed and compared through Kaplan-Meier survival estimate between the two group.Results:Part 1 The median survival time of all patients was 7.4(0.1-17.3)years,and the 1-year,3-year,5-year,and 10-year survival rate wrer 92.5%,89.6%,88.2% and 82.1 respectively. The median survival time of DM patients was 8.3(0.1-17.3)years,and the 1-year,3-year,5-year,and 10-year survival rate wrer 92.1%,89.4%,88.1% and 81.8% respectively.The median survival time of CADM patients was 5.0(0.1-17.6)years,and the 1-year,3-year,5-year,and 10-year survival rate wrer 94.1%,90.0%,88.3% and 84.8% respectively.We found that the survival rate of two groups were not different significantly by Log-rank test(x2=0.05,p=0.8081). Log-rank test showed patients with hyperpyrexia(χ2=80.01,p=0.0000),heliotrope rash(χ2=7.00,p=0.0081), severe muscle weakness(≤2 degree)(χ2=50.37,p=0.0000) or dyspnea(χ2=8.04,p=0.0046) as the first symptom,and with old age(≥60 years old)(χ2=37.21,p=0.0000),delayed therapy(90 days after pathogenesis)(χ2=16.51,p=0.0003),ANA(+)(χ2=7.29,p=0.0069),low of serum albumin(<30g/L)(χ2=18.64,p=0.0000),high of serum LDH(≥300 U/L)(χ2=7.53,p=0.0061),respiratory infections(χ2=18.96,p=0.0000), Interstitial pneumonia(χ2=26.50,p=0.0000),malignant tumor(χ2=8.81,p=0.0000) lived a short time.The COX regression showed old age(HR=2.01,95%CI:1.99～2.31,p=0.041), delayed therapy(90 days after pathogenesis)(HR=1.99,95%C:1.98χ2.01, p=0.043),hyperpyrexia(HR=3.73,95%CI:1.13χ12.29,p=0.030),complicated with malignant tumor(HR=5.43,95%CI:1.11～3.90,p=0.018) or ILD(HR=2.62,95%CI:1.13～6.06,p=0.024) were the independent risk factors of the prognosis.Part 2 There were 102 patients with chronic ILD and 20 patients with acute ILD.The prevalence of cough and dyspnea of acute ILD patients was higher than the one of chronic ILD patients statistically(χ2=31.81,p=0.000 andχ2=32.34,p=0.000).The average value of PaO2 in acute ILD patients was statistically lower than the in chronic ILD patients(t=4.03,p=0.0003).The difference of the level of muscle strength and creatase were not significant statistically between the two groups.We found dry cough was the predict factor of the occurrence of acute ILD in PM/CADM patients(OR=21.771,95% CI:1.5-324.5,p=0.025)through Logistic regression analysis.The prognosis of patients with acute ILD was worse than the one of patients with chronic ILD statistically(χ2=15.59,p=0.0001).Conclusion1.The long survival rate was no different significantly between the two groups.Old age, delayed therapy, hyperpyrexia,interstitial pneumonia and malignant tumor were risk factors influencing survival.2.Cough was the predict factor of the occurrence of acute ILD in PM/CADM patients.3. The prognosis of patients with acute ILD was worse than the one of patients with chronic ILD.