| Objectives: 1.To investigate the clinical and laboratory characteristics of anti-melanoma differentiation associated gene 5 positive(MDA5+)polymyositis/dermatomyositis(PM/DM)patients by comparing the clinical characteristics and laboratory indexes of MDA5 + and MDA5 negtive(MDA5-),as well as anti-synthetase autoantibodies positive(ASA +)PM/DM patients.2.To explore risk factors of mortality in PM/DM patients through follow-up study.Methods: 1.Medical records of 744 PM/DM patients hospitalized in our hospital from January 2002 to November 2016 were retrospectively collected.Patients were divided into MDA5 + group,MDA5-group as well as ASA + group according to different myositis specific antibodies(MSA).Demographic information,clinical characteristics,laboratory examinations and chest high resolution computerized tomography(HRCT)information were analyzed.2.Four-hundred ninety patients with follow-up data were divided into death and survival group according to their survival status.Risk factors affecting the survival rate of polymyositis/dermatomyositis were analyzed.Results: 1.Compared with MDA5-group,the proportion of non-specific Systemic symptoms(such as fever,weight loss),typical skin involvement(such as heliotrope,Gottron’s sign,V-rash,shawl sign,mechanic hands,skin ulcer),arthralgia,interstitial lung disease(ILD)and rapidly progressive interstitial lung disease(RPILD)in anti-MDA5+group significantly increased,while the proportion of concomitant tumors,other autoimmune diseases,muscle weakness and dysphagia significantly decreased.2.Compared with ASA+ group,the proportion of weight loss,typical skin involvement(photoallergy,heliotrope,Gottron’s sign,V-rash,shawl sign,skin ulcer),arthralgia and RPILD in anti-MDA+ group significantly increased,while the proportion of concomitant tumors and other autoimmune diseases significantly decreased.3.The cumulative survival rate of MDA5 + group was significantly lower than that of MDA5-group and ASA + group.The 1-year,5-year and overall cumulative survival rates were 74%,59%,59% in MDA5 + group,92%,86% and 70% in MDA5-group,and 96%,91% and 70% in ASA + group(p < 0.001).Cox regression analysis showed that anti-MDA5 antibody,concomitant tumors,dysphagia,elevated serum aspartate aminotransferase(AST)and erythrocyte sedimentation rate(ESR)levels were independent risk factors for poor prognosis of PM/DM.Conclusions: The main clinical characteristics of MDA5+ patients are typical rash,severe lung involvement and relatively mild muscular lesions,which are significantly different from ASA+ patients in clinical manifestations and prognosis.The prognosis of patients with positive anti-MDA5 antibody,tumor,dysphagia and high level of AST and ESR is poor,so it is necessary to follow up closely and formulate individualized drug regimens.Objective: We aim to investigate the risk and prognostic factors of rapidly progressive interstitial lung disease(RPILD)in dermatomyositis(DM)patients with anti-MDA5 antibody.Methods: Medical records and follow-up data of anti-MDA5 antibody positive DM(anti-MDA5+DM)patients hospitalized in the rheumatology department of our hospital from January 2010 to December 2017 were collected.Patients were divided into RPILD and non-RPILD group according to comprehensive evaluation of clinical and imaging.Logistic regression models were adopted to identify the risk factors of RPILD.Comparisons of serum ferritin(FET)levels before and after treatment in patients with different prognosis were analysed to explore the value of FET in the evaluation of curative effect.Follow-up study was performed to explore the risk factors of death within 6 months in anti-MDA5 antibody positive DM patients.Results: Ninety-five cases of anti-MDA5+DM patients were included,with 40 cases(42.1%)in RPILD group and 55 cases(57.9%)in non-RPILD group.There were no significant differences in gender,DM proportion,skin and muscle manifestations between the two groups.The onset age of RPILD group was older than that of non-RPILD group,and the incidence of weight loss was higher than that of non-RPILD group.Serum level of ferritin(FET),erythrocyte sedimentation rate(ESR)and C-reactive protein(CRP)in RPILD group were significantly higher than those in non-RPILD group,while the peripheral blood CD3+T cell count was significantly lower than that in the non-RPILD group.Comparisons before and after treatment indicated that serum FET level was correlated to the prognosis of interstitial lung disease(ILD),while ESR and CRP had no significant changes before and after treatment.Multivariate logistic regression showed that elderly onset age,weight loss,as well as high serum FET and ESR were independent risk factors for the development of RPILD in anti-MDA5+DM patients.Multiple cox regression showed that the risk of death within 6 months significantly increased in RPILD patients with infection and high serum ferritin level.The cumulative survival rate was significantly lower in patients with higher FET level(FET>1230ng/ml)than those with lower FET level(FET≤1230ng/ml)(P=0.025).Conclusions: RPILD is more likely to occur in anti-MDA5+DM patients with elderly onset age,weight loss,as well as high serum ferritin and ESR,closely monitoring of these patients is of vital importance.The risk of death within 6 months significantly increased in RPILD patients with infection and high serum ferritin,intensive treatment should be performed with the exclusion of infection in these patients.Objectives: 1.In the retrospective analysis section,we explored clinical and serological characteristics of Dermatomyositis(DM)related rapid progressive interstitial lung disease(RPILD)through analyzing clinical characteristics and laboratory indicators.2.In the follow-up study,we aimed to investigate the clinical value of checking serum chitinase-3-like-1 protein(YKL-40)and matrix metalloproteinase 7(MMP-7)levels in anti-MDA5 antibody positive dermatomyositis(anti-MDA5+DM)patients.Methods: 1、A total of 105 DM patients with anti-MDA5 antibody were retrospectively collected from August 2003 to December 2017 in our Hospital.Forty-four sex and age matched healthy people were included as controls.Demographic information,clinical features,laboratory examinations,pulmonary function and HRCT information were collected by electronic medical record system.Patients were further divided into RPILD group and non-RPILD group according to whether RPILD occurred.Univariate analysis was performed to investigate the differences of clinical characteristics and laboratory examinations,as well as YKL-40 and MMP-7 levels between the two groups.2、Twenty-one patients with serum one month after treatment were divided into stable/improved group and aggravated group according to the prognosis of interstitial lung disease(ILD).The changes of serum YKL-40 levels before and after treatment were compared.3、Patients were divided into death or survival group according to 6-month survival.Risk factors affecting 6-month survival rate of anti-MDA5 positive DM-RPILD were analyzed.Results: Forty one out of 105 anti-MDA5+DM patients had RPILD at the time of serum sample collection(39.0%).Serum YKL-40[48.75(27.30,89.23)vs 14.94(10.49,22.22)ng/ml,P < 0.0001] and MMP-7 [3.76(2.54 5.32)vs 2.90(2.46,4.43)ng/ml,P =0.042]levels in anti-MDA5+DM patients were significantly higher than those in healthy controls.Serum YKL-40 levels were significantly higher in anti-MDA5+DM patients with RPILD compared with those without [80.3(28.7,107.7)vs 42.4(26.6,73.1)ng/ml,p=0.011 ].No significant difference was observed in MMP-7 level between the two groups [3.41(2.39,5.24)vs 4.06(2.59,5.48),p=0.508].One month after treatment,patients with aggravated ILD had increased YKL-40 levels[25.4(17.7,40.4)vs 43.6(32.3,76.1)ng/ml p=0.031],while those with stable/improved ILD had decreased YKL-40 levels[73.2(25.8,118.3)vs 26.7(13.0,63.6)ng/ml,p=0.007].Higher serum levels of ferritin and YKL-40,as well as lower peripheral CD3+T cell counts,were independently associated with poorer prognosis.Kaplan–Meier survival curve showed that the 6-month survival rate in patients with high serum YKL-40 level(>80 ng/ml)was significantly lower than that in patients with low YKL-40 level(≤80 ng/ml),(67% vs 89%,p < 0.01).Conclusions: YKL-40 can be useful as an indicator for the occurrence of RPILD,and correlates with severity of ILD and poor prognosis in anti-MDA5+DM patients.Closely monitoring and intensive treatment are suggested in anti-MDA5+DM patients showing high level of YKL-40,especially levels >80 ng/ml. |
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