Clinical Study Of Adult Dermatomyositis With Subcutaneous Calcification

Objective:(1)To identify clinical features and risk factors in adult dermatomyositis(DM)with calcinosis.(2)To analyze treatment,survival rate and prognostic factors of adult DM patients with calcinosis.Methods:(1)Four hundred and eighty patients’ clinical data were collected.The correlation between calcinosis and no calcinosis in adult DM were calculated by t test,χ2 tests and Mann-whitney U test.Multiple factors logistic regression model was established to analysis independent factors of adult DM with calcinosis.(2)A retrospective analysis was performed on 25 patients who admitted into our hospital.T test,Mann-Whitney U test,Fisher test,Kaplan-Meier survival analysis and Log-rank test were used for statistical analysis.Results:(1)Calcinosis occurred in 480 patients with DM in 22 cases.The incidence rate of adult DM with calcinosis was 4.6%.(2)Calcinosis was most common on the extremities and trunks.(3)Patients with calcinosis had a longer disease duration[48(24,120)monthsVS10(3,24)months,U=1993,P=0.000)]and more myothenia(95.5%VS 76.9%,x2=4.192,P=0.038),panniculitis(9.1%VS 0.4%,x2=19.025,P=0.001),perlungual erythematosus(22.7%VS 5.89%,x2=9.558,P=0.011),skin ulcer(50.0%VS 5.2%,χ2=62.213,P=0.000),raynaund’s phenomenon(27.5%VS 8.1%,χ2=5.642,P=0.035).The anti-NXP2 antibody(27.3%VS 3.9%,χ2=24.080,P=0.000)and anti-MDA5 antibody(31.8%VS 12.9%,χ2=6.347,P=0.021)were more frequently found in patients with calcinosis.(4)Multiple factors logistic regression showed that anti-NXP2 antibody(OR=10.899,95%CI 2.593,45.816,P=0.001),long diseases duration(OR=1.105,95%CI 1.008,1.021,P=0.000)and skin ulcer(OR=31.585,95%CI 10.683,93.387,P=0.000)were the risk factors of adult DM with calcinosis.(5)52%of patients received steroid combined one immunosuppressive agent,48%of patients received steroid combined more than one immunosuppressive agent.40%of patients were treated with therapies targeted at calcinosis.(6)The average survival time was 92+120 months.The survival rate at 1,5 and 10 years from the diagnosis respectively were 95.8%,91.3%,91.3%.The causes of death in two patients respectively were lymphoma and infection.Patients associated with cancer and negative MSA were higher mortality.(7)Patients with calcinosis improvement had lower baseline calcification VAS score,higher rate of V sign and remission in 3 months.Remission in 3 months was the protective factor of patients with calcinosis improvement.Conculusion:(1)The incidence rate.of adult DM with calcinosis was 4.5%in our cohort.(2)Patients with calcinosis were distinct clinical subset of adult DM.(3)Long disease duration,skin ulcer and anti-NXP2 positive were independent risk factors in adult DM with calcinosis.(4)Adult DM patients with calciniosis were lower mortality,but patients of calcinosis associated with cancer and negative MSA were higher mortality.(5)Remission in 3 months was the protective factor of patients with calcinosis improvement,which indicated that aggressive treatment for patients with calcinosis at very early stage of disease may improve the outcome.