Clinical Features Analysis Of Antineutrophil Cytoplasmic Antibody-associated Vasculitis With Lung Involvement

BackgroundANCA-associated vasculitis(AAV)is a systemic,necrotizing vasculitis with few or no immune deposits.It mainly invades small vessels(i.e.capillaries,venules or arterioles),but it also has the possibility of involving small arteries.Its classification mainly includes microscopic polyangiitis(MPA),granulomatous polyangiitis(GPA)and eosinophilic granulomatosis with polyangiitis(EGPA).AAV is often secondary to infections,autoimmune diseases and tumors.The most common clinical manifestations are lung-kidney involvement.In addition to non-specific manifestations such as fever,fatigue and weight loss,the involvement of digestive system,nervous system,limb joint muscles,skin and ear,nose and throat can also be seen.The exact incidence is not yet clear,and its clinical manifestations are often atypical.Some patients come to the hospital for diagnosis and treatment due to pulmonary symptoms or abnormal chest imaging,which is prone to misdiagnosis and missed diagnosis.Objective To investigate the clinical characteristics of pulmonary involvement in patients with microscopic vasculitis,and to explore the relationship between disease activity and clinical characteristics and treatment prognosis.Improve the awareness of medical workers,especially respiratory physicians,and the level of diagnosis and treatment of the disease.ObjectiveTo investigate the clinical characteristics of pulmonary involvement in patients with microscopic vasculitis,and to explore the relationship between disease activity and clinical characteristics and treatment prognosis.Improve the awareness and diagnosis of the disease among medical workers,especially those in respiratory department.MethodsThe clinical data of 196 patients with pulmonary involvement with ANCA-related vasculitis treated in the First Affiliated Hospital of Zhengzhou University from January 2013 to January 2018 were retrospectively analyzed.According to the naming of systemic vasculitis at the Chapel Hill Conference in 2012 and the diagnostic criteria for systemic vasculitis established by the American Rheumatism Society in 1990,the patients were divided into three groups: MPA,GPA and EGPA.The clinical characteristics of the patients were compared.Imaging,laboratory examination,treatment and clinical remission.SPSS 23.0 software was used for statistical analysis and chart drawing.The measurements that conform to normal distribution are expressed by X+s,and the comparison among independent samples is made by one-way ANOVA.The counting data are tested by 2 test,and the test level is alpha=0.05.ResultsThere were 102 males and 94 females.The age of onset was(47.39 ±14.27)years.There were 82 patients with pulmonary symptoms.The common clinical symptoms were fever,cough,phlegm,fatigue,chest tightness,asthma,digestive tract symptoms,nervous system symptoms,myalgia,lower limb edema,joint pain,hemoptysis,rash and so on.The chest imaging manifestations were ground glass opacity,mediastinal lymph node enlargement,plaque consolidation,fibrous cord opacity,pleural thickening,pulmonary nodules,emphysema,etc.The other 62 cases were nasal sinus CT scans.It suggests sinusitis.Extrapulmonary organs involved were kidney,cardiovascular system,nervous system,ear,nose and throat,digestive system in turn.Comparing the data of different types of patients,we can see that the frequency of abnormal symptoms in chest CT images is different.There were significant differences in laboratory tests of leukocyte,erythrocyte,hemoglobin,creatinine,glomerular filtration rate,erythrocyte sedimentation rate,urinary protein and occult blood.The anti-MPO antibody values of MPA patients were significantly higher than those of the other two groups,while the anti-PR3 antibody indexes of GPA patients were significantly higher than those of the other two groups.The majority of MPA patients were p-ANCA positive,while the c-ANCA positive rate of GPA patients was higher.After hormone and immunosuppressive therapy,185 patients achieved short-term clinical remission.Eight patients chose to give up treatment because of the deterioration of their condition.Four patients died of severe pulmonary infection and multiple organ failure.ConclusionThe clinical features of pulmonary involvement of AV are atypical,and there is no significant gender difference.It is common in middle-aged and elderly people,and can involve single or multiple systems.There were significant differences in clinical manifestations,imaging and laboratory examinations among AAV patients of different types.P-ANCA,c-ANCA,anti-PR3 and anti-MPO antibodies were significantly associated with different classifications.GC combined with immunosuppressive agents can significantly improve clinical symptoms in a short time.