Clinical Analysis Of 94 Cases Of Juvenile Dermatomyositis And Risk Factors Of Prognosis Of Juvenile Dermatomyositis

ObjectiveJuvenile dermatomyositis(JDM)is a rare autoimmune paediatric disease and it is the most common inflammatory myopathy of childhood.It is characterized by proximal musle weakness and pathognomonic skin rashes.In this study we performed a retrospective study to assess the clinical manifestations and outcomes of 94 patients with JDM in Peking Union Medical College Hospital and to explore the risk factors of the prognosis of JDM.MethodsNinety-four patients with JDM were studied retrospectively between 1990 and 2016.Analysis the general data,clinical features,laboratory data and outcomes of the 94 cases by SPSS 24.0.Classified the patients into monophasic course of disease and poor prognosis.Logistic regression analysis was performed to explore the risk factors of prognosis.ResultsOverview of the 94 patients:The incidence of CADM among the 94 cases was 4.26%.There were 2 cases dided of intersititial lung disease with severe lung infections.Forty-four patients achieve the duration of follow up more than 3 years.Thirty-one patients(70.45%)achieved clinical remisson and the medium time reached this end point was 7.00(4.50-17.50)months.Among the 31 patients,15 patients have achieved clinial recovery.The average time reached this end point was 50.46months.Twelve patients had chronic course of disease.One patient infected by EB virus and progressed to hemophagocytic syndrome.The calcinosis and pulmonary affected are more common in patients with poor prognosis.JDM affected pulmonary is the risk factor of poor prognosis.ConclusionsJDM can affect mutiple organs.The presenting features and the course of disease are variable.More than 70%patients can achieve clinical remission JDM affected pulmonary is the risk factor of poor prognosis.Patients with JDM should have more tests to find the pulmonary affected in time.