A Retrospective Clinical Study Of Aneurysms Caused By Rheumatic And Immune Diseases

Part ? A Retrospective Clinical Study of Aneurysms Caused by Rheumatic and Immune DiseasesBackground Rheumatic and immune diseases have a diverse pathogenesis and an extensive area of involvement.Besides commonly seen lesions in the joints,connective tissue,skin,mucosa,vascular lesions are an important aspect that cannot be overlooked.Aneurysms are the most common manifestation of arterial disease and are a form of pathological dilatation diseases caused by injury or lesions to arterial walls.Patients with severe conditions may even experience a ruptured aneurysm,resulting in shock or even death.Currently,etiology studies suggest that aneurysm formation is a result of the cumulative effects of participation of multiple inflammatory factors,environment,genetics,hemodynamics,and other factors.As the incidence of aneurysms caused by rheumatic and immune diseases is relatively low,the primary disease is often masked by the aneurysm,if only treating the aneurysm will tend to result in postoperative recurrence or even rupture,endoleaks,and other serious complications.Objective The aim of this study was to retrospectively analyze the incidence and clinical characteristics of aneurysms caused by rheumatic and immune diseases in order to provide more accurate and reasonable diagnoses and treatments in future clinical practice.Methods The medical records of hospitalized patients in Changhai Hospital affiliated Naval Medical University(Former Second Military Medical University)who were diagnosed with aneurysms from January 1,2001 to December 31,2017 were used as the study participants.Previous medical history,clinical symptoms and signs,laboratory results,and the imaging and pathological results of patients were used to screen for aneurysms caused by rheumatic and immune diseases and to analyze the etiological composition,gender,and age differences,aneurysm distribution status,and imaging characteristics.These factors was analyzed and compared with aneurysms caused by non-rheumatic causes in age,disease course,systemic and local symptoms,involvement of various systems,and laboratory markers.The data were analyzed by SPSS version 19.0 software package.Results 1.There were 9174 aneurysms cases,including 165 cases of aneurysms caused by rheumatic and immune diseases.The composition of aneurysms caused by rheumatic and immune diseases in overall etiology was approximately 1.80%,of which 76 were males and 89 were females,with an incidence ratio of more females than males(2.68% vs.1.30%,x~2=22.707,P<0.001).The incidence of multiple aneurysms in rheumatic and immune diseases group was higher than that in non-rheumatic group(33.33% vs.18.09%,x~2=25.101,P<0.001).The age of onset and development of aneurysms in rheumatic and immune diseases group(34.76±14.16 years;40.35±15.96 years)was higher than that of the group with congenital arterial dysplasia(29.44±14.14 years,t=4.189;36.16±14.45 years,t=3.145;P<0.001)and lower than those of the group with hypertension/ hyperlipidemia/ atherosclerosis(53.42±10.22 years,t'=-16.832;61.08±12.44 years,t'=-16.576;P<0.001),the group with trauma/ iatrogenic injury(48.26±16.98 years,t'=-8.994;48.94±16.91 years,t=-5.274;P<0.001),and the group with infection by pathogenic microorganisms(53.87±17.00 years,t=-8.069;54.44±17.05 years,t=-5.461;P<0.001).The disease course of rheumatic and immune diseases group was longer than those of the group with trauma/ iatrogenic injury(3.0 vs.0.5 years,U=8257.5,P<0.001)and the group with infection by pathogenic micro organisms(3.0 vs.0.5 years,U=1400.5,P<0.001)and shorter than that of the group with hypertension/ hyperlipidemia/ atherosclerosis(3.0 vs.5.0 years,U=507263.5,P<0.001).2.The proportion of patients with systemic symptoms was higher in rheumatic and immune diseases group(46.67% vs.12.61%,x~2=163.797,P<0.001).Among these systemic symptoms,fatigue was the most common symptom(43 cases,26.06%).In terms of local symptoms and involvement of other systems,the proportion of vascular murmurs,lesions in the skin / mucosa and joints / muscles in rheumatic and immune diseases group was higher than that in non-rheumatic disease group,respectively(33.94% vs.14.22%,x~2=50.613;13.33% vs.0.07%,x~2=937.256;3.64% vs.0.08%,x~2=145.013;P < 0.001).In terms of laboratory test results,hemoglobin(Hb)levels in patients from rheumatic and immune diseases group were lower than those in patients from the non-rheumatic disease group(117.14±22.19 vs.124.46±20.00 g/L,t=-4.651,P<0.001),while C-reactive protein(CRP)and Erythrocyte sedimentation rate(ESR)levels in patients from rheumatic and immune diseases group were higher than those in patients with non-rheumatic aneurysms(15.20 vs.7.31 mg/L,U=41492.5;28 vs.15 mm/H,U=40556.0;P<0.001).3.Takayasu arteritis(TA)was the most common disease(96 cases,58.18%)in rheumatic and immune diseases group.There were differences in etiological composition between genders(x~2=50.937,P<0.001),of which TA(31 cases,40.79%),Beh?et's disease(BD)(16 cases,21.05%),and chronic periaortitis(CP)(15 cases,19.74%)were more common in males and TA(65 cases,73.03%),systemic lupus erythematosus(SLE)(13 cases,14.61%),and giant cell arteritis(GCA)(4 cases,4.49%)were more common in females.Involvement of the abdominal aorta was the most common in patients with aneurysms caused by rheumatic and immune diseases(80 locations,29.30%).There were also certain differences in the distribution of different diseases in other affected arteries.4.Pathology and imaging results had an important significance in the clinical diagnosis and differential diagnosis,but comprehensive judgment based on the patient's condition,clinical signs and symptoms,and laboratory tests is still required.5.Glucocorticoids(GC)and immunosuppressants were still the mainstay drugs used for the treatment of aneurysms caused by rheumatic and immune diseases.Besides emergency situations,such as an excessively large aneurysm volume or rupture,it was recommended that surgical intervention be conducted after arterial inflammatory responses were controlled or treatment with the aforementioned drugs can managed while undergoing surgical treatment.If necessary,multi-disciplinary diagnoses and treatments should be recommended.ConclusionThe incidence of aneurysms caused by rheumatic and immune diseases in females was relatively higher than that in males.There were differences in age of onset,course of disease and when aneurysms occur was compared with other etiologies.Aneurysms caused by rheumatic and immune diseases showed a higher proportion of multiple aneurysms,systemic symptoms,local vascular murmurs,and involvement of skin/ mucosa and joints/ muscles.In laboratory test results,patients in rheumatic and immune diseases group had lower Hb levels and higher CRP and ESR levels.In terms of etiological composition,TA was the most common disease.There were differences in the etiological composition and aneurysm distribution between genders.Involvement of the abdominal aorta was the most common in patients with aneurysms caused by rheumatic and immune diseases.Pathology and imaging results had important significance in clinical diagnosis and differential diagnosis,while GC and immunosuppressants were still the mainstay drugs for treatment.In clinical practice,multi-disciplinary diagnoses and treatments should be conducted based on the patient's condition,clinical signs and symptoms,and various supplementary examination results.Part ? Takayasu Arteritis Complicated with Aneurysms: A Retrospective Clinical Study of 96 CasesBackground Takayasu arteritis(TA)is a chronic vasculitis that mainly involves the aorta and its major branches.TA occurs mostly in young women aged less than 40 years in all races of the world,but is especially common in Asian populations.TA incidence varies considerably among countries or regions.Some studies have shown that the annual TA incidence is 0.9/million people,0.4-1.0/million people,2.0/million people and 4.7/million people in the United States,Germany,Norway and the United Kingdom,respectively,while it can reach 40/million people in Japan.The clinical manifestations of TA mainly include systemic symptoms,local vascular inflammation,arterial stenosis or occlusion-induced ischemic symptoms,arteriectasia,formation and rupture of aneurysms,and other extravascular symptoms,among which aneurysm rupture is the most dangerous.Some studies have shown that TA complicated by an aortic aneurysm is a potentially life-threatening factor.Objective The previous part of our study has found that TA is the most common cause of rheumatic and immune aneurysm.In this part of the study,we aim to retrospectively analyze TA aneurysms in terms of occurrence patterns,clinical features,disease activity,and angiographic classification types,etc.Methods The medical records of hospitalized patients in Changhai Hospital affiliated Naval Medical University(Former Second Military Medical University)who were diagnosed with TA from January 1,2001 to December 31,2017 were used as the study participants.We divided them into two groups,aneurysm group and non-aneurysm group,according to complicating with aneurysms or not.We retrospectively analyzed the general informations,clinical symptoms and signs,aneurysm characteristics,other system involvement,laboratory and imaging results.We classified the patients according to the criteria developed by Numano group in 1996,and we used Indian Takayasu Clinical Activity Score(ITAS 2010)to assess the disease activity of the patients.The data were analyzed by SPSS version 19.0 software package.Results 1.In this study,there were 397 TA patients,including 96 patients with aneurysms.The overall incidence of TA aneurysms was around 24.18%.The average age when aneurysms appeared in the patients was 34.06±12.47 years.The average median time to onset of aneurysms was 2.0(1.0,3.0)years.The ratio of males to females with TA aneurysms was 1:2.1.Male as a risk factor for TA aneurysm(OR=0.258,95%CI 0.147-0.452,P<0.001)and the rate of aneurysms in males was significantly higher than that in females(48.44% vs.19.52%,x~2=24.486,P<0.001).2.TA aneurysms were most commonly found in the abdominal aorta(39 locations,25.00%),successively followed by the carotid artery(24 locations,15.38%),the ascending aorta(23 locations,14.74%),the descending thoracic aorta(22 locations,14.10%),and the renal artery(12 locations,7.69%).Compared with patients in the non-aneurysm group,patients in the TA aneurysm group had a higher rate of pain(63.54% vs.36.88%,x~2=21.076,P<0.05),vascular murmur(51.04% vs.36.88%,x~2=6.070,P<0.05),cardiac complications(31.25% vs.15.95%,x~2=10.797,P<0.05),gastrointestinal symptoms(15.63% vs.5.32%,x~2=10.746,P<0.05),and an average level of CRP in laboratory tests(13.30 vs.7.55mg/L,U=5582.5,P<0.05).On the contrary,the patients in the TA aneurysm group had a lower rate of pulse weakening or disappearance(59.38% vs.76.08%,x~2=10.084,P < 0.05)and neurological symptoms(59.38% vs.73.09%,x~2=6.491,P<0.05).3.The most common angiographic classification type in all the TA patients was type I(155 cases,39.04%),while the most common type in the aneurysm group was type V(30 cases,31.25%).Differing from the non-aneurysm group,the TA aneurysm group had a lower proportion of type I(20.83% vs.44.85%,x~2=17.641,P<0.05)and a higher proportion of type IIa(11.46% vs.4.32%,x~2=6.532,P<0.05).4.Glucocorticoids(GC)are still the first-line drug to treat TA aneurysms.There is non-uniformity between the assessment of the clinical disease activity and the actual disease activity.Since the goal of drug therapies is to provide effective control over the patient's condition,it is necessary to use GC and/or other immunosuppressants in the perioperative period.Conclusion The overall incidence of TA aneurysms was about 24.18%.Male is a risk factor for TA aneurysm.The incidence of aneurysms in males was significantly higher than that in females.The most common site of the aneurysms was the abdominal aorta.Compared with patients in the non-aneurysm group,patients in the TA aneurysm group had a higher rate of pain,vascular murmur,cardiac complications and gastrointestinal symptoms,and average level of CRP in laboratory tests.On the contrary,the patients in the TA aneurysm group had a lower rate of pulse weakening or disappearance and neurological symptoms.The most common angiographic classification type in the aneurysm group was type V.Compared with the non-aneurysm group,the TA aneurysm group had a lower proportion of type I and a higher proportion of type IIa.GC is still the first-line drug therapy.There is non-uniformity between the assessment of the clinical disease activity and the actual disease activity.It is necessary to use GC and/or other immunosuppressants in the perioperative period to effectively control the patient's condition.