| Objective: To analyze the clinical features and therapeutic effects of patients with systemic sclerosis(SSc)and interstitial lung disease(ILD),and to provide reference for the diagnosis and treatment of SSc-ILD.Methods:To collect clinical data of 128 patients with SSc who were admitted to the Department of Rheumatology and Immunology,the First Affiliated Hospital of Guangxi Medical University from July 6,2012 to October30,2018,including general conditions,clinical manifestations,laboratory tests,High-resolution chest CT(HRCT)and so on.Divided into SSc-nonILD group and SSc-ILD group,and then divided into localized SSc-ILD(lcSSc-ILD)group and diffuse SSc-ILD(dcSSc-ILD)group according to the skin involvement range.For the patients with common interstitial pneumonia(UIP)and non-specific interstitial pneumonia(NSIP),the recombination and stable group were added according to the changes of chest HRCT score after treatment,and their different characteristics and treatment outcomes were retrospectively analyzed.And related risk factors.Result:1.Of the 128 patients with SSc,55 were male and 73 were female.Theage was(13-75)years old,the median age was 47 years,the course of disease was from January to 30 years,and the median duration was 9.5 months.Among them,SSc-ILD patients There are 70 cases(54.7%).The most common clinical manifestation of SSc-ILD was Raynaud's phenomenon(85.7%),followed by post-exercise shortness of breath(42.9%),cough(40.0%),and joint swelling and pain(38.6%).Seven patients with SSc-ILD were treated with hormone alone,and the rest were treated with hormones(5-40 mg/d)and immunosuppressive agents(48 patients with cyclophosphamide(cumulative amount 1.0 g-9.8 g)and 17 patients with methotrexate.There were 14 cases of thalidomide,10 cases of tripterygium glycosides,9 cases of azathioprine,5cases of hydroxychloroquine,3 cases of mycophenolate mofetil,2 cases of leflunomide,and 1 case of cyclosporine.2.The median duration of disease was 13 months in the SSc-ILD group,significantly longer than the 6 months in the SSc-nonILD group(P<0.05);cough in the SSc-ILD group compared with the SSc-nonILD group(40% vs22.4%)ESR(29(19-52)vs 18(11-31))mm/h,hypersensitive C-reactive protein(CRP)(9.6(5.2-15.6)vs 6.5(2.3-11))mg/ L,the positive rate of anti-nuclear antibody(ANA)was significantly increased(17.1% vs 1.7%),but the positive rate of ANA nucleoli type(17.1% vs 36.2%)was significantly lower,P<0.05.3.The chest HRCT of SSc-ILD patients was most common with ground glass(50 cases)and mesh shadow(36 cases).The CRP of the lcSSc-ILD group before treatment was 12.9(7.0-51.5)mg/L,which was significantly higher than that of the dcSSc-ILD group(8.6(4.3-13.3))mg/L(P<0.05).Before and after treatment,the lcSSc-ILD group There was no significant difference in chest HRCT scores between the dcSSc-ILD group and the dcSSc-ILD group(P>0.05).4.The median duration of the SSc-UIP group was 60.0 months,which was significantly longer than the median course of 11.0 months in the SSc-NSIP group(P<0.05).Compared with the SSc-NSIP group,the SSc-UIP group had cough(57.6%).Vs 24.3%),complement C3 level [0.9(0.8-1.0)vs 1.1(1.0-1.2)],complement C4 level [0.3(0.2-0.4)vs 0.2(0.2-0.3)],chest HRCT score [(11.0(8.0-14.0)vs 6.0(4.0-8.0)]were significantly increased,both P<0.05.The chest HRCT score of SSc-UIP group was still higher than that of SSc-NISP group after treatment for 6 months [12.0(9.5-16.5)vs 6.0(4.0-8.0)].However,there was no significant change in chest HRCT score before and after treatment(P>0.05).5.Before treatment,compared with the SSc-ILD stable group,SSc-ILD plus reconstituted patients with post-operative airlift(52.2% vs 38.3%)were more obvious,and IgA levels were higher than the former(P<0.05).There were no significant changes in the laboratory parameters of the two groups after treatment.Logistic regression analysis showed that the course of disease(OR=0.021,P=0.016),ANA homogeneity(OR=0.109,P=0.043),complement C4(OR=8682.110,P=0.034),post-activity shortness of breath(OR=4.116),P =0.030)is an independent risk factor for SSc-ILD.Immunoglobulin IgA levels were a risk factor for exacerbation of SSc-ILD(OR=0.423,P=0.039).Conclusion:1?The incidence of ILD in patients with SSc is 54.7%.The patients with SSc-ILD have a long course of disease,common cough symptoms,higher ESR and CRP,common homogenization of ANA,and common HRCT in the chest.2?There was no significant difference in ILD between lcSSc-ILD and dcSSc-ILD.Patients with SSc-UIP had longer course of disease,more commoncough symptoms,higher levels of CRP,C3,C4,and more serious ILD.Six months after hormone and immunosuppressive therapy,SSc There was no significant change in ILD in patients with UIP and SSc-NISP.3 ?The course of disease,post-activity shortness of breath,ANA homogeneity,complement C4,is a risk factor for SSc-ILD.IgA levels are a risk factor for aggravation of patients after SSc-ILD treatment. |
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