| Objective:To investigate the clinical manifestations, new developments in assessment, prognosis of juvenile dermatomyositis(JDM) and evaluation the clinical application of muscle histopathology, to improve the level of diagnosis and treatment.Materials and methods:Using the dermatomyositis diagnostic criteria of Bohan and Peter, to select the dermatomyositis children who did muscle biopsy.Investigation the clinical manifestations, laboratory data, treatment and prognosis of patients with JDM. This was a retrospective analysis of11children who diagnosed of JDM between2002and2012and followed up at our department.Those patients were analyzed retrospectively, to find the correlation among muscle biopsy,MRI and clinical manifestations.Results:Among the11JDM patients,6were boys,5were girls, the sex ratio was6:5. The median age at onset of JDM was6years and4months. There were no positive family history,no significant difference between the regional and seasonal.5patients onset of chronic (5/11,46%).3patients onset of the JDM was subacute (3/11,27%, the acute onset of the JDM was3patients (3/11,27%). Erythra was the most common first symptom(5/11,45%), the first manifestation of2patients were fever and muscle weakness(2/11,18%). Each had1cases of edema and subcutaneous scleroma patients(1/11,9%). All the people had different degree of muscle weakness.8cases had Gottrons’sign (73%),10cases had heliotrope rash(91%).4cases (36%) had repeatedly low fever. Most of them combined respiratory tract infection(5/11,45%), such as acute respiratory tract infection(3/11,27%), pneumonia(2/11,18%).2patients had abnormal signal of the muscles detected by MRI. It mainly expressed for high signal on T2WI, on the early stage. The main performance of muscle histopathology were endothelial cell swelling, narrowing and obliteration of the vessel lumen, and perivascular inflammation(8/11,73%), perifascicular atrophy, and muscle fiber degeneration/regeneration(10/11,91%). Electromyography of10patients were muscle abnormal.91%patients had increased myocardial enzyme, AST(73%), LDH(82%), HBDH(82%), CK(55%) and CK-MB(45%).9patients were treated with corticosteroids, children could combine with application immunosuppressive agents.2children were given C-type globulin support on acute phase. Amony the11JDM patients, no body had malignant tumor or interstitial lung disease.3patients had a significantly elevated erythrocyte sedimentation rate.8patients (73%) discharged in good and stable condition,2patients(18%) discharged themselves, One patient(9%) died from massive gastrointestinal hemorrhage.Conclusion:The average age of onset about seven years old. The early symptoms of JDM is skin rashes. The clinical manifestations are variety, weakness of proximal muscles, pathognomonic skin rashes and Muscle enzyme increasing are main performance. Most of them combine respiratory tract infection. The cardiovascular system, digestive system can also be involved. Few children will develop malignant tumor or interstitial lung disease.,Muscle pathologic examination is the gold standard for JDM. MRI can show inflammation of active JDM, as a high signal intensity on T2WI. The short-term the flip restore sequence (STIR) scanning is more sensitive. MRI can provide an assessment of disease activity and damage. MRI can detect the disease early, but the lack of specificity, can’t make a definite diagnosis. It can provide the accurate positioning for muscle biopsy, and be helpful for dermatomyositis of early diagnosis. Muscle biopsy is needed, when clinical manifestation is not typical, muscle pathologic examination can make a definite diagnosis of JDM and identify other causes of myopathy. The main performance of muscle histopathology are muscle fiber degeneration/regenerations, narrowing and obliteration of the vessel lumen, perivascular inflammation. On the advanced stage, muscle fiber atrophy, fat and connective tissue infiltration and replaced. The remaining number of muscle is the main factor that affect the recovery degree of muscle strength. Muscle fibers necrotizing as a serious damage, the remaining number of muscle obviously reduced, has a poor prognosis. The prognosis of mild necrotizing muscle is better. Corticosteroids are the first choice drug, acute period can give vein shock treatment. Combined application of immune inhibitors can reduce the dosage of hormone. The acute phase also can give c-type globulin support treatment. Critically ill patients can use plasma exchange. Early diagnosis and treatment can improve the prognosis, and reduce the complications. Most children have a benign prognosis. A few patients have muscle atrophy, calcify, joint contracture and so on. Critically ill patients can died. |
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