ABSTRACT
<p><b>OBJECTIVE</b>To explore the clinical and laboratory features and the prognosis of juvenile dermatomyositis (JDM) complicated with interstitial lung disease (ILD).</p><p><b>METHOD</b>Data of 39 cases of JDM complicated with ILD hospitalized in Beijing Children's Hospital from January 2005 to December 2011 were collected. The clinical features, laboratory data and prognosis of these children were analyzed.</p><p><b>RESULT</b>Of the 39 cases studied, 16 were boys, and 23 girls. The average age of onset was 5.6 years, and 61.5% of the patients' age of onset (24 cases) was under 6 years. Rashes (17 cases, 43.6%), simultaneous eruption of rashes and muscle weakness (14 cases, 35.9%), fever (4 cases, 10.1%), or muscle weakness (3 cases, 7.7%) were common initial symptoms of the disease. Only 51.3% of the patients (20 cases) had the symptoms of respiratory system, but (24 cases) 61.5% were complicated with that of the gastrointestinal system; (27 cases) 69.2% had at the same time electrocardiographic and echocardiographic abnormalities. The chest high resolution computed tomography (HRCT) showed cord or band-like shadows in their lungs of more than half of the cases (25 cases, 64.1%), and other changes included ground glass-like shadow (10 cases, 25.6%), net and lineation-like shadow (9 cases, 23.1%), nodular change (5 cases, 12.8%). The patients complicated with lung essential infiltration accounted for as high as 71.8% (28 cases). These imaging changes were largely seen on both dorsal sides of their lungs. Severe patients also had mediastinal emphysema, pneumothorax, pneumorrhagia or aerodermectasia. Twenty-four patients underwent pulmonary function examination, and 62.5% of the patients' pulmonary function (15 cases) was abnormal. The fatality rate of the cases studied was 10.1%.</p><p><b>CONCLUSION</b>The imaging changes of patients suffering from JDM with ILD were often more severe as compared to the clinical symptoms, and were often complicated with damages to other systems and organs. The prognosis of those patients was poorer than others. Patients with JDM especially at a younger age of onset and with various organ damages should be examined with chest HRCT examinations as early as possible.</p>
Subject(s)
Child , Child, Preschool , Female , Humans , Male , Dermatomyositis , Diagnosis , Drug Therapy , Glucocorticoids , Therapeutic Uses , Immunosuppressive Agents , Therapeutic Uses , Lung , Diagnostic Imaging , Pathology , Lung Diseases, Interstitial , Diagnosis , Drug Therapy , Methotrexate , Therapeutic Uses , Muscle Weakness , Diagnosis , Epidemiology , Prognosis , Respiratory Function Tests , Retrospective Studies , Tomography, X-Ray ComputedABSTRACT
Objective To investigate the long-term prognosis an d recorery of children with juvenile rheumatoid arthritis(JRA).Methods The cases diagnosed JRA in our hospital over the period of 1988～1992 we re followed up for the conditions of disease,the deteriorated joints,the treatme nt and the living conditions of patients.Results Ninty-six ca ses were followed up(involving male 66 cases,female 29 cases,the mean age of on set 8.21?3.17 years )except for a case who died of lymphoma.There were 44 cases with systemic JRA ,2 cases of them died after 7-years onset and 23 cases developed severe destructive arthritis.There were 38 oligoarthritis cases,27 cases of them stiu had active disease during 10-year following-up and 9 cases were diagnosed sacroiliitis.There were 13 polyarthritis and 3 cases of them were RF positive, who had developed severe destructive arthritis.Conclusions The prognosis of juvenile rheumatoid arthritis is not desirable,especially in systemic JRA,whose prognostic factors are related to age of onset,the lasting of fever,the markers of phlegmasia activity and the condition of systemic involvement and treatment.Oligoarthritis about 30 % may develop into ankylosing spondylitis.The probability of destructive arthritis is hi gher in polyarthritis with more RF positive and poor prognosis.