| Background Dermatomyositis is a rare autoimmune disease which mostly involves the skin and muscles.The disease causes characteristic skin damage and myositis manifesting muscle pain,swelling,tenderness and weakness.Commonly,the proximal muscles of limbs are involved.However,it can also affect the esophageal muscles,heart muscles,respiratory muscles,lungs and kidneys in severe cases.Although some cases are related to infections and malignant tumors,the cause and mechanism of the disease have not yet been elucidated to date.Clinically,the management is still challenging for severe cases.So,it is helpful for guiding the management of dermatomyositis to conduct a comprehensive analysis on the clinical data of patients with dermatomyositis.At present,the assessment of disease severity is mainly based on the degree of serum muscle enzyme elevation,severity of muscle damage and complications.Recently,some studies have shown that some myositis autoantibodies are closely related to the clinical phenotypes and prognosis of dermatomyositis,such as anti-MDA5 antibody,anti-TIF-1 antibody,anti-NXP-2 antibody,anti-EJ antibody,anti-Mi-2 antibody and anti-Jo-1 antibody.Therefore,it is important for evaluating the clinical prognosis and guiding the clinical treatment of patients to develop the detection of these autoantibodies.Research Purposes The aim of this study is to improve the clinical diagnosis and treatment of dermatomyositis by analyzing the clinical features and clinical efficacy of 447 cases of DM patients.In addition,we explore the clinical significance of detection of myositis autoantibodies in the diagnosis,treatment and assessment of prognosis of dermatomyositis. Experimental materials and methods We collect and analyze the clinical data of 447 cases of DM inpatients from 1997 to2017 in the department of dermatology and venerology,the first affiliated hospital of Anhui medical university.The clinical data and blood samples of 42 cases of DM patients are collected.16 myosistis autoantibodies are then examined by western blotting.Results There were 447 patients,including 286 cases with single hospitalization and 161 with multiple hospitalizations.A total of 351 DM patients were excluded after repeated information,and there were 33 child patients aged from 2 to 15 years with an average onset of 8.88±3.56 years,and 318 adult patients aged from 16 to 87 years with an average onset of 50.93±15.43 years.There were 137 male patients and 214 female patients,and the ratio of male to female patients was 1:1.562.The first symptom was skin lesion in 251 cases(252 / 351,71.5%),muscle damage in 12 cases(12 / 351,3.4%).260(59.1%)patients had extensive skin lesions,and 194(44.3%)patients had Gottron's papules in 438 patients.There were 150 cases(37.2%),53 cases(13.2%),92cases(22.8%)and 108 cases(26.8%)with normal,mildly damaged,moderately damaged and severely damaged muscle strength of upper limbs respectively in 333 patients.There were 53 cases(16.2%),126 cases(43.9%),85 cases(25.9%)and 46cases(14%)with normal,mildly damaged,moderately damaged and severely damaged muscle strength of lower limbs respectively in 310 patients.70 patients had normal muscle strength of all limbs.Of the 428 patients,there were 39 cases(9.1%)with muscle swelling,112 cases(26.2%)with muscle tenderness,69(16.1%)with dysphagia,and 27(6.3%)with cough due to drinking water.Fever occured in 52 cases(11.6%)and blood system damage in 308 cases(68.9%)of 447 patients.42 cases(12%)were accompanied with malignant tumors in 351 patients.There were 147 cases(33.3%)with elevataed serum ALT level(51 ~ 1142 U / L)in 442 patients,272 cases(61%)with elevataed serum AST level(36 ~ 1785 U / L)in 446 patients,350 cases(82%)with elevataed serum LDH level(270 ~ 3564 U / L)in 427 patients,254 cases(60.8%)with elevataed serum CK level(137 ~13657U / L)in 418 patients,155(57.8%)cases with elevataed serum CKMB level(27 ~ 873 U / L)in 268 patients,168 cases(80.4%)cases with elevataed serum ?-BDH level(185 ~ 1732 U / L)in 209 patients.Among 279 patients,161 cases(57.7%)were positive for ANA and only 1 case was positive for anti-Jo-1 anibodies.222 of 248 cases(89.5%)showed typical change of myogenic lesion in electromyogram examination.Muscle biopsies were performed in 212 patients,and there was swelling of muscle fibers in 62 cases,degeneration and necrosis of muscle fibers in 13 cases,dissolution and partial rupture of muscle fibers in 62 cases,atrophy of muscle fibers in 6 cases,and lymphocyte infiltration in 117 cases.CT examination was performed in 120 cases,and pulmonary interstitial changes were observed in 53 cases(44.2%).Among 447 patients,405(90.6%)were treated with glucocorticoid of routine dosage except methylprednisolone pulse therapy in 1 case.The initial dose of glucocorticoid therapy for patients with the highest CK level >300U/L ranged from 0.11 to 2.5 mg/kg.d predinisone(only 1 case with initial dose 0.11 mg/kg.d with an average of 1.26 ± 0.42 mg / kg.d.The initial dose range in child group(? 15years)was 0.38 ~ 2.5 mg / kg.d with an average of 1.24 ± 0.49 mg / kg.d,0.11 ~ 2.12mg/kg.d with an average of 1.06±0.46mg/kg.d in adult group(>15 years).The control dose range of glucocorticoid is 0.3~2.5mg/kg.d,an average of1.44±0.69mg/kg.d.The control dose range in child group was 0.38 ~2.5mg/kg.d with an average of 1.44±0.52mg/kg.d,0.3~2.5mg/kg.d with an average of 1.39±0.37mg/kg.d in adult group.Of the 447 patients,367 cases(82.1%)got better and 80 cases(17.9%)gave up treatment or died because of worse state of illness.Pulmonary infection complicated in 51 patients(11.4%).2.The male patients had higher percentages of abnormal function of swallowing in esophageal barium meal examination,interstitial lung disease and malignant tumors(49%,20.7% and 17.1%)compared with female patients(21.9%?8.3% and 8.4%),all P<0.05.While the percentage of blood system damage in female patients(78.1%)was higher than that in male patients(54.3%)(P< 0.05).The percentage of associated malignant tumors in DM patients aged > 60 years(21.4%)was significantly higher than that in DM patients aged ?60 years(7.9%)(P <0.05).The serum LDH level in patients with ?3 level muscle strength of lower limbs(709±653)was significantly higher than that in patients with > 3 level muscle strength(452±345)(P< 0.05).3),while there were no statistical significance in serum CK level between these two groups.The percentage of pulmonary infection in patients >60 years(22.8%)was significantly higher than in patients ?60 years(9%)(P<0.05).The higher the dosage of glucocorticoid,the higher the percentage of decrease in serum CK level(P<0.05).3.Among 42 DM patients with myositis,36 cases(85.7%)were positive for anti TIF antibody,19 cases(45.2%)positive for anti Ro52 antibody,12 cases(28.6%)positive for anti Ro52 antibody,5 cases(11.9%)positive for anti NXP2 antibody,4 cases(9.5%)positive for anti OJ antibody,4 cases(9.5%)positive for anti-Ku antibody,4 cases(9.5%)positive for anti-SAE1 antibody,cases(9.5%)positive for anti-MDA5 antibody,cases(9.5%)positive for anti-Mi-2 antibody,3 cases(7.1%)positive for anti-PL7 antibody,2 cases(4.8%)positive for anti-Mi-2 antibody,only 1 case(2.4%)positive for anti-EJ antibody,anti-SRP antibody,anti-Jo1 antibody,an i-PM / Scl 75 antibody and anti-PM / SCL 100 antibody.One,two and three kinds of anti-myositis antibodies were found in 19 cases(45.2%),11 cases(26.2%)and 4 cases(9.5%)respectively.The percentages of malignant tumors in patients with anti-TIF? antibody was significantly higher than that in patients without anti-TIF? antibody(P<0.05).The percentages of LID in patients with anti-TIF? antibody was significantly lower than that in patients without anti-TIF? antibody(P<0.05).All 4 patients with positive anti-MDA5 antibody had complicated interstitial lung disease.Moreover,3 of 4 patients showed unresponse to clinical therapy.All patients showed myositis in histology,and 3 patients had severe myalgia in 5 DM patients with anti-NXP2 antibody.Conclusion1.The average age of onset in child patients and adult DM patients was 8.88 ±3.56years?50.93 ±15.43 years respectively.The female patients are dominant for DM.A majority of patients have the involvement of blood system.The male DM patients are prone to develop abnormal function of swallowing in esophageal barium meal examination,interstitial lung disease and malignant tumors compared to female patients.While the female patients had more blood system damage than male patients.The DM patients aged > 60 years are susceptible to develop malignant tumors compared to DM patients aged ?60 years.The CK level is normal in about 40% of DM patients.The positive rate of anti-Jo1 antibody is very low in DM.The serum levels of LDH in patients with severely impaired muscle strength were significantly higher than those with mildly impaired muscle strength.The mean hormonal doses for the control of the disease in the children and adult groups were 1.44 ± 0.52 mg / kg.d and 1.39 ± 0.37 mg /kg.d.2.The anti-MDA5 antibody are associated with complication of interstitial lung disease in DM.Additionally,anti-TIF-?antiblody is suggestive of paraneoplastic DM. |
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