Factors influencing prognosis of 90 polymyositis and dermatomyositis patients / 第三军医大学学报

ABSTRACT

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.