The Clinical Analysis Of 47 Cases Of Systemic Sclerosis

Objective:This study was performed to analyze the clinical characteristics and examination results of Systemic sclerosis(SSc),aiming to enhance the understanding of SSc and provide references for the early diagnosis and treatment,delaying organ damage and improving prognosis.Methods:In this retrospective observational study,folders of 47 patients with SSc seen in the Dermatology and Rheumatology department of Zhujiang Hospital affiliated to Southern Medical University between October 2017 and October 2019 were retrospectively analyzed,including general information,skin lesions,physical examinations,laboratory and auxiliary examinations.SPSS 24.0 statistical software was used to analyze and process the data.Results:(1)Forty-seven patients with SSc had been evaluated,among which were 7 males and 40 females,31 limited systemic sclerosis(LcSSc)and 16 diffuse systemic sclerosis(DcSScs).The mean age was 50.79±12.80 years,mean onset age was 45.87± 14.24 years and mean course of disease was 4.68±8.09 years.The average age at onset of DcSSc and males was higher than that in LcSSc and females,the course of LcSSc is longer than that of DcSSc.(2)The most common initial symptom was Raynaud’s phenomenon(RP),it was found in 31 patients(66.0%).The incidence of skin lesion was found as followed:91.5%cutaneous sclerosis,85.1%cutaneous swelling,61.7%oral skin changes,59.6%masklike facies,55.3%poikiloderma,46.8%fingertip pitting scars,42.6%telangiectasias,27.7%nail or perionychium changes,23.4%nose changes,23.4%skin atrophy,12.8%digital ulcers(DUs).There was a high positive rate of RP accompanied with telangiectasia,fingertip pitting scar,positive autoantibodies(Scl-70、SSA、Ro-52)and increased erythrocyte sedimentation rate(ESR)respectively.(3)There were 28(59.6%)cases of musculoskeletal involvement,24(51.1%)cases of gastrointestinal involvement,2(4.3%)cases of pulmonary arterial hypertension(PAH),33(70.2%)cases of interstitial lung disease(ILD),16(34.0%)cases of abnormal electrocardiogram(ECG),19(40.4%)cases of abnormal heart color doppler ultrasound.The onset age of males and DcSSc with lung involvement were higher than that in females and LcSSc.(4)The positive rate of anti-scl-70 antibody in LcSSc was higher than that in DcSSc,while the increased transaminase and high-sensitivity C-reactive protein(HSCRP)and abnormal ECG were lower than that in DcSSc,showing the difference were statistically significant.(5)The positive rate of masklike facies and pulmonary involvement in the anti-scl-70 antibody positive group was significantly higher than that in the anti-scl-70 antibody negative group,while the ECG and increased immunoglobulin were lower than that in the anti-scl-70 antibody negative group.The average age and onset age of the anti-CENP-B antibody positive group were higher than that of the anti-CENP-B antibody negative group.(6)There was a significant incidence of nail or perionychium changes accompanied telangiectasias,oral skin changes accompanied poikiloderma,ingertip pitting scars or shortening distal segment accompanied poikiloderma,DUs or oral skin changes.When it turns to the association between organs involvement and skin lesion,it shows that telangiectasias accompanied pulmonary involvement,ingertip pitting scars or shortening distal segment or oral skin changes accompanied gastrointestinal involvement,oral skin changes accompanied abnormal ECG got a higher incidence.Conclusion:(1)Occurs at a younger age,SSc is more prevalent in women than in men,also LcSSc is more common.(2)With a variety of skin lesions,RP is often the earliest cutaneous sign of SSc.Cutaneous sclerosis,masklike facies,poikiloderma,fingertip pitting scars,DUs,telangiectasias,hemorrhage under the nailfold are the characteristic cutaneous manifestation of SSc.Besides,RP accompanied with positive autoantibodies,increased ESR may imply the high disease activity.(3)When it involves visceral organs,patients may occur the clinical symptom as well.It seems that pulmonary involvement occurs in women and LcSSc earlier than that in males and DcSSc.(4)Anti-scl-70 antibody is more common in LcSSc,while aminotransferase and HSCRP elevation and ECG changes are more prone to happen in DcSSc.Further more,patients with positive anti-scl-70 antibody are more likely to appear masklike facies and lung involvement,while ECG changes and elevated immunoglobulin are more often occur in those without anti-scl-70 antibody.