Clinical Characteristics And Survival Analysis Of Patients With Eosinophilic Granulomatous Polyangiitis

Background:Eosinophilic granulomatosis with polyangiitis is one of the rarest systemic necrotizing vasculitides predominantly affecting small-middle vessels,which characterized by asthma and eosinophilia.In our country,several descriptive series have been published to understand the main characteristics of this disease,but each with limited patient numbers and followup durations.There is a lack of research on the clinical characteristics,treatment,follow-up and survival prognosis of EGPAObjective:1.EGPA patients in Peking Union Medical College Hospital was included in this study to describe the baseline of the cohort.Survival analysis was performed to assess the risk factors for death.2.Compare the clinical features,organ involvement,laboratory indicators,pathological manifestations and treatment according to their ANCA status.To investigate the effect of ANCA on the phenotype of disease.3.To analyze the clinical features of patients with large and middle-sized arterie involvementCompare the clinical features,organ involvement,laboratory indicators,pathological manifestations and treatment according to with or without large and medium-sized arteries involvementMethods.A retrospective study of EGPA patients in the Peking Union Medical College Hospital who satisfied the American College of Rheumatology criteria and/or confirmed by pathology was conductedl from January 2000 to December 2015.Follow up points to December 2017.Result:1.181 cases of EGPA were diagnosed,including 110 males and 71 females,with a male to female ratio of 1.5:1.The age was 15-80 years and the median age was 45 years(31,59).2.The most common organs involved were lung in 147 cases(81.2%),ENT involvement in 126 cases(69.6%),skin involvement in 86 cases[47.5%],nervous system involvement in 84 cases(46.4%),and digestive system involvement in 56 cases.(30.9%),renal involvement in 40 patients(22.1%),cardiac involvement in 25 patients(13.8%),venous thromboembolism in 12 patients(6.6%],and large and middle-sized arteries in 18 patients(9.9%).3.Patients with pulmonary involvement ranged from 6 to 540 months from onset to diagnosis,with a median time of 86 months;patients with no involvement of the lung ranged from 2 to 240 months from onset to a diagnosis,and a median time of 24 months.P=0.02.Of the 35 patients without pulmonary involvement,19 were confirmed by biopsy.4.38 patients were positive for ANCA test,among which p-ANCA and MPO-ANCA were the most common positive,with 30 cases(78.9%)and 25 cases(65.8%),respectively.Fever,peripheral nerve involvement,renal involvement,platelet value,serum creatinine value,elevated ESR,24-hour urinary protein>1.0g,pathological manifestations of angiitis were more frequently found in ANCA-positive patients than in ANCA-negative patients.In patients with ANCA-negative disease,the involvement of the heart and digestive system is more common than the ANCA-positive patients.ANCA status was not significantly associated with death.5.Patients aged>65 years(at the time of diagnosis),myocardial involvement,and severe gastrointestinal involvement were independent risk factors for death.6.Patients with large or middle artery involvement were more likely to have joint swelling,pain,skin,and venous thromboembolism than patients with no or large middle artery involvement.Patients without large or middle-sized arteries involvement were more likely to have fever,asthma,or lung infiltrates than those with 1 arge and medium-sized arterie involvementConclusion1.Asthma is the most common symptom in EGPA patients,but asthma in Chinese EGPA patients is much lower than that in European and American countries.When the clinical manifestations of patients are not specific,pathological biopsy is a good means for early diagnosis.2.The characteristics of EGPA patients differ according to their ANCA status.ANCA status was not associated with death,3.Through survival analysis,it is once again confirmed that the FFS score has important clinical value for the evaluation of EGPA prognosis4.EGPA can lead to large and medium-sized arteries involvement,including coronary,cerebral and extremities arteries.When patients have premature myocardial infarction,cerebral infarction,accompanied by asthma and eosinophil increase,EGPA may be considered when conventional treatment is not effective,and early diagnosis should be sought.