| Research BackgroundAntineutrophil cytoplasmic antibody(ANCA)-associated vasculitis(AAV)is a systemic small-vessel vasculitis,usually manifested as multi-organ damages such as lung and kidney which are the most common and fatal target organs.Other organ-threatening injuries also occur in skin,nerve system and digestive system and other systems,which result in variable clinical manifestations in AAV patient and immensely increase the difficulty in disease diagnosis and treatment leading to a higher mortality rate.AAV,as small-vessel vasculitis,was rarely connected to cardiovascular injury.In recent years,researchers from home and abroad have found out that cardiovascular damage in AAV is not only leading to higher morbidity,usually presenting as pericarditis,coronary vasculitis and valve lesion,but also closely related to unfavorable prognosis.Therefore,exploring the clinical characteristics of AAV patients with cardiovascular involvement and analyzing the related clinical risk factors for occurrence of AAV cardiovascular damage are helpful for clinicians to reduce misdiagnosis and mistreatment;therefore,prognosis of disease will be improved.ObjectiveThe research is aimed at exploring the related clinical risk factors for occurrence of AAV cardiovascular involvement,via the clinical characteristics,laboratory and imaging data,treatments and prognoses of AAV patients.Subjects and MethodsClinical information was retrospectively selected and collated from 268 AAV patients of hospitalization in Nanfang Hospital from January,2006 to July 2017.159 cases out of 268 AAV patients that were confirmed with cardiovascular damage were allocated to a case group while the remaining 109 patients were served in a control group.Clinical,laboratory,therapeutic,prognoses and imaging data were comparably analyzed.Baseline data recorded for each patient included age,gender,medical history,clinical manifestations,laboratory measures,electrocardiogram,imaging data,treatment and prognoses.Birmingham vasculitis activity score(BVAS)of every patient was calculated at the time of onset of illness.All the data were analyzed by SPSS20.0.Results1 Clinical characteristics of AAV with cardiovascular damageThe overall incidence of cardiovascular damage out of 268 AAV patients was 159cases(59.3%),including 74 males(46.5%)and 85 females(53.5%).The age distribution of all patients ranged from 12 to 83 years old while the average age of cases was(55.97±15.50)years old,with males aged(57.18±14.51)years old and females aged(54.93±16.33)years old.100 patients(62.9%)had the history of hypertension while 39 cases(24.5%)got diabetes.Cases with smoking history accounted for 20.8%.The average BVAS was(13.81±6.51)while first diagnosed.The main cardiovascular symptom was edema(35.8%)and breathing difficulties(25.2%).33 cases(20.8%)suffered from chest tightness or chest pain.Only 4 patients(2.5%)complained of palpitation.100 out of 159 patients(62.9%)had heart failure in the course of the disease.Among 100 patients,44 cases(27.7%)were classified as NYHA class Ⅰ,26 cases(16.4%)were class Ⅱ and 23(14.5%)were class Ⅲ,while class Ⅳ in 7 patients accounted for 4.4%.Auxiliary examinations such as electrocardiogram,echocardiography and image examination revealed that valve lesion was the most common cardiovascular manifestation,accounting for about 73.40%in a series of 159 patients,characterised by mitral regurgitation(68.60%),aortic regurgitation(64.20%)and tricuspid regurgitation(57.20%).What’s more,27 cases(17.00%)were caused by mitral valve calcification,36 cases(22.60%)were manifested as aortic calcification while 2 and 5 patients(1.30%and 3.10%)presented with mitral stenosis and aortic stenosis respectively.Meanwhile,there were 51 out of 159 patients(32.10%)manifested as arrhythmia while pericardial effusion occurred in 39 patients(24.50%),with 29 cases(18.20%)presented with pulmonary arterial hypertension and myocardial damage in 24 cases accounted for 15.10%.Moreover,4 patients(2.50%)suffered from myocardial infarction during the course of disease while 8 cases(5.0%)were diagnosed according to coronary angiography.Apart from cardiovascular involvement,multi-organ damages occurred generally in AAV patients such as kidney injury,lung damage,nervous and digestive damage as well as ear-nose-throat(ENT)and skin involvement.In addition,serious complications such as septicemia and septic shock came up times to times while 10 patients were found malignant tumors concurrently.Higher values of serum NT-proBNP was detected in AAV patients with cardiovascular involvement with average of(12059.58±13651.21)pg/ml while myocardial enzymes were also elevated in a large number of patients including lactate dehydrogenase(LDH)(374.53±1218.25)U/L,hydroxybutyrate dehydrogenase(HBDH)(258.17±473.91)U/L,creatine kinase(CK)(172.47±752.27)U/L and cardiac creatine kinase(CKMB)(18.29±53.51)U/L.In addition,creactive protein(CRP)increased in 80.5%of AAV cardiovascular involvement patients and anemia had the same proportion.And 91.8%of cases presented with hypoproteinemia.The average level of serum creatinine(SCr)was(385.77±+327.65)μmol/L and the concentration of serum urea nitrogen(BUN)was(20.89±47.20)mmol/L.The values of triglyceride(TG)and cholesterol(CHOL)were in the normal range.About 126(79.2%)AAV patients with cardiovascular involvement were found MPO-ANCA positive and 29(18.2%)were PR3-ANCA positive through method of enzyme linked immunosorbent assay(ELISA).P-ANCA positive was detected in 113(71.1%)patients while C-ANCA positive was 47(29.6%)by indirect immunofluorescence assay(IFA).The most common positive autoimmune antibodies was Antinuclear Antibody(ANA)in this research(24.5%)with the positive rates in the remaining antibodies ranged from 0 to 10.7%.Among 159 patients,101 of them(63.5%)received glucocorticoid[1 mg/(kg·d)]with immunosuppressor such as cyclophosphamide in combination as basic treatment.However,51 cases(32.1%)relied only on glucocorticoid while 7 patients(4.4%)didn’t receive any basic treatment.For other therapeutic methods,the application for hormone stosstherapy(0.5～1.0 g/d,for 3 days)accounted for 50.3%while 15.7%were treated with intravenous immunoglobulin.Plasmapheresis was adopted in 47 cases and in the meanwhile 55 patients had to relied on hemodialysis.Most of the patients achieved remission except 12 cases(7.5%)of death.2 Comparison between case group and control group(with/without cardiovascular involvement)and analysis of risk factors.2.1 Comparison of general and clinical data Compared with pateints in control group,patients with cardiovascular involvement showed a significant statistical difference in age,history of hypertension,BVAS,the incidence of hydrothorax,serum creatinine level and positive rate of C-ANCA(P<0.05)while damage in other systems,complications and treatments displayed no significant difference.2.2 Multicariate regressions Results revealed that age(OR,1.020;95%CI,1.003-1.037;P=0.018),BVAS(OR,1.064;95%CI,1.019-1.112,P=0.005),SCr(OR,1.002;95%CI,1.001-1.003,P=0.001),C-ANCA(OR,0.332;%CI,0.177-0.624,P=0.001),P-ANCA(OR,0.509;95%CI,0.267-0.970;P=0.040)were independent predict factors for cardiovascular damage in patients with AAV.Conclusions1.Cardiovascular damage caused by AAV occurs frequently with various clinical manifestations,which is not attached enough importance so far.Therefore better management guidelines of diagnosis standard,treatment and risk assessment are in desperate demand.2.Advanced age,high Birmingham vasculitis activity score,increased creatinine level might be the potential related risk factors of the occurrence of cardiovascular damage in AAV patients while ANCA positive may play a protective role.3.There’s no relation between cardiovascular involvement and high death rate in AAV patients. |
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