Clinical Features And Prognosis Of Microscopic Polyangiitis With Pulmonary Involvement

ObjectiveTo explore the clinical features and risk factors of prognosis in MPA(Microscopic Polyangiitis)patients,so as to improve the understanding of the disease.Methods81 cases with confirmed diagnosis of MPA from January 2010 to December2018 were collected from my hospital,data includes patients’ general situation、clinical symptoms、BVAS、laboratory examination、pulmonary imaging、pathology examination、treatment、prognosis.The clinical symptoms、laboratory examination、treatment and prognosis were compared between patients with pulmonary involvements and patients with non-pulmonary involvements.According to the outpatient or telephone follow-up to record the prognosis.Statistical analysis was done by SPSS 24.0.Results1.General situation: The 81 cases which including 40 males and 41 females met my criteria were enrolled.MPA usually occurred in middle-aged,the mean(±SD)age was 56.6±15.0,the mean(±SD)time from onset to diagnosis was 6.10±10.87 months.51 cases appeared pulmonary involvements,24 cases of non-pulmonary involvements.2.Respiratory symptoms: The first symptom is fatigue(29.6%).Kidneys and lungs are the most often affected organs.The main manifestations of 81 cases were cough(45.7%),sputum(39.5%),shortness of breath(24.7%),edema(34.6%),bubbles in urine(27.2%).Cough,sputum,shortness of breath,hemoptysis,blood in mucus phlegm,fever,BAVS were significantly higher in patients with pulmonary involvements(P<0.05).3.Laboratory examination: The MPO-ANCA was significantly higher in patients with pulmonary involvements(P=0.003).No significant differences were found between patients with pulmonary involvement and patients non-pulmonary involvements in leukocyte count,neutrophil count,platelet count,hemoglobin concentration,C-reactive protein,serum creatinine,blood urea nitrogen,serum albumin,urine erythrocyte count,24-hour urine protein quantification,complement C3,Complement C4,immunoglobulin A,immunoglobulin G,immunoglobulin M.4.Pulmonary imaging: The most common sign was ground glass shadow(35.09%).Other signs were fibrous cord shadow,pleural effusion,emphysema,etc..5.pathology examination:Histopathological examination was performed in 22 patients,all of which were renal biopsy.According to the pathological classification of Berden/EUVAS,there were 10 cases of crescentic type,7 cases of sclerosing type,1 case of focal type and 1 case of mixed type.6.Treatment and prognosis : Systemic corticosteroid is the most common medication treatment(60.49%),followed by corticosteroid combined with immunosuppressant(29.63%).Up to December 2018,69 of 81 patients died with a mortality rate of 17.28%.7.Prognostic factors: the mean survival time of the patients in this study was72.697 months(95%ci 65.572-79.822),and the cumulative survival rate at 1,3 and 5years was 87.3%,82.2% and 80.0%,respectively.Log-rank test indicated that lung injury(P=0.04),ESRD(P=0.000),initial need for dialysis(P=0.000)were risk factors for survival(P<0.05).Gender(P=0.72),age(P=0.223),ANCA titer(P=0.207),CRP(P=0.848),serum albumin(P=0.457),large amount of proteinuria(P=0.385),and BVAS score(P=0.092)had no statistical significance for survival.COX regression analysis indicated that ESRD(P=0.000)and initial need for dialysis(P=0.001)were independent factors affecting the survival rate of MPA patients,and lung injury(P=0.189)was not statistically significant.ConclusionMPA usually occurred in middle-aged,and its clinical manifestations are lack of specificity.The most common sign in chest imaging is ground glass shadow.ESRD and initial need for dialysis were independent factors affecting survival in MPA patients,which were independent of gender,age,ANCA titer,CRP,serum albumin,large amount of proteinuria and BVAS score.