Clinical Characteristics And Risk Factors Of 170 Cases Of Systemic Sclerosis

Background:Systemic sclerosis(SSc),so called scleroderma,is a kind of connective tissue diseases characterized by skin and visceral fibrosis and microangiopathy.Systemic sclerosis is a rare disease which carries high motality and disability rate.The pathogenesis of systemic sclerosis remains unclear,however,the progression and prognosis of the disease are heterogeneous among patients,some of whom progress rapidly and have the worse therapeutic efficacy.Nowadays,people try to deepen the insight of SSc through dividing into different groups according to clinical characteristics and compare their difference.Raynaud’s phenomenon(RP)is a common initial symptom and microangiopathy of systemic sclerosis,which could appear a few years even more than decades before the definite diagnosis of SSc.As the disease progresses,physical sign that affect the quality of life of SSc patients and increase the disability rate will appear,such as digital ulcers,gangrene,shorten of phalanges.Vasculophathy is also associated with other important organs involvement,such as interstitial lung disease(ILD).We analyzed the clinical features,risk factors and prognosis of a series of vasculophathy and pulmonary involvement of SSc patients to deepen our understanding of SSc.Objective:Observe the clinical characteristics of raynaud’s phenomenon in SSc;Analyse the clinical characteristics and risk factors of SSc patients who was accompanied by digital ulcers,microangiopathy(gangrene or shorten of phalanges),ILD and the prognosis of SSc.Methods:Clinical information was collected from systemic sclerosis patients of hospitalization in Nanfang hospital from October 2002 to November 2018,who met the diagnostic criteria of SSc.The baseline data,clinical manifestations,auxiliary examination,treatment and so on were recorded,and we collected the prognosis information in November 2019.Chi-square test,independent sample t test and other statistical methods were used to anlysis the data.Results:1.Basic information:among the 170 cases of SSc patients,there were 59 cases males,111 cases females;48 cases lcSSc,122 cases dcSSc,and males were prone to be dcSSc(50/59);the average age was 49.4±12.5 years old,disease duration was 1(0.7,4)years,follow-up time was 1.8(0.2,4.8)years.2.Age onset of the disease:there was a lower prevalence of anti-Scl 70 positive(OR 0.341;95%CI 0.167,0.695)and higher prevalence of anti-Ro 52 positive(OR 2.885;95%CI 1.118,7.444),ILD(OR 2.100;95%CI 1.108,3.979)and PAH(OR 11.200;95%CI 1.299,96.541)in patients who were older than 50 years old at disease onset.3.RP:patients who were accompanied by RP(113 cases)were more likely to be ANA positive(93/101),and the positive rate was up to 92.1%.Patients who were accompanied by RP were more likely to appear digital tip ulcers or pitting scars(OR 2.7;95%CI 1.106,6.591)and pleural thickening(OR 3.044;95%CI 1.248,7.423),but less likely to appear articular involvement(OR 0.447;95%CI 0.232,0.859).As for the the patients whose diease duration were more than 2 years,patients who were accompanied by RP were easier to be presence of ILD.5.Digital ulcers:patients who appeared digital ulcers were prone to be ANA positive(97/81 cases),anti-Scl 70 positive(56/98 cases),but less likely to be hyperlipidmia(16/38 cases vs 65/87 cases).Multivariable analysis showed that anti-Scl 70 positive was the risk factors of digital ulcers(OR 5.234;95%CI 1.803,15.196),while the hyperlipidmia was the protect one(OR 0.177;95%CI 0.071,0.441).4.Microangiopathy:females were common among the patients who were presence of microangiopathy(20/23 cases),multivariable analysis suggested that joint contracture(OR 4.529;95%CI 1.303,15.742)and anti-scl 70 positive(OR 5.496;95%CI 1.029,29.361)were the risk factors of the occurrence of microangiopathy,while the calcium channel agent(OR 0.169;95%CI 0.034,0.851)was the protective one.5.ILD:Multivariable analysis suggested that anti-Scl 70 positive(OR 2.659;95%CI 1.158,6.105),older than 50 years at disease onset were the risk factors of ILD(OR 2.463;95%CI 1.137,5.334).6.Follow-up and prognosis:Among the 134 patients who were followed up,14 patients developed irreversible damage of vital organs(brain,heart,lung,kidney),of whom 64.3%were dcSSc,and 10 death were all dcSSc.Among the patients who got the autoantibody testing,the death all were anti-Scl 70 positive.COX analysis suggested that anti-scl 70 antibody(HR 5.978;95%CI 1.745,20.474)were risk factors for symptom recurrence or exacerbation.Conclusions:In this study,females were common among SSc patients,males were prone to be dcSSc.Anti-Scl 70 positive was the risk factor for the occurrence of ILD,digital ulcers,microangiopathy,symptom recurrence or exacerbation,indicating that anti-Scl 70 positive means a poor prognosis.The risk factors of the occurrence of microangiopathy were joint contracture,while calcium channel agent was the protective factor.