Clinically Amyopathic Dermatomyositis With Interstitial Lung Disease:a Retrospective Study Of33Patients

Background:Polymyositis/dermtomyositis is one of the classical connective diseases.Clinically amyopathic dermatomyositis(CADM) is the rare phenotype, with a high incidence of interstitial lung disease.The aim of the study is to assess the clinical features and prognostic factors for CADM with ILD.Methods:Thirty three patients with CADM in the second affiliated hospital of Zhejiang University from year2009-2014, who has a proof of interstitial lung disease.,were included in our study. We collect the clinical manifestation, signs,pulmonary function, therapy,outcome.Then analyze the data in SPSS software,using Independent-Samples T Test Cox regression to get the survival time and hazard ratios.Results:In the33patients,the ratio of male to female was4:29, Overall survival time was10days to4years. Almost All patients had Gottron rash and interstitial pneumonia.Apart form6patients,the rest of the patients had the manifestation of cough,fever or short of breath.14patients died in2months,while19patients stay stable.The death group had a higher rate of skin ulcer, fever, carcino embryonic antigen and IgA elevation.Patients not taking SMZ had a higher mortality rate than taking one(OR=9.46).In the died group,there were no significance in survival time between taking hormone pulse therapy and not.Conclusion:CADM with ILD had a low positive rate of ANA and anti Jo-1antibody.Fever,skin ulceration,elevated IgA are the predictive factor for poor prognosis.Taking SMZ during their courses can help improve the survival rate.While the hormone pulse therapy had no benefit on prolonging the survival time.