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Objective@#To investigate the clinical featuresand related factors of anti-neutrophil cytoplasmic antibody associated vasculitis (AVV) with interstitial lung disease (ILD), and to explore the high-resolution computed tomography (HRCT) of the chest features between different anti-neutrophil cytoplasmic antibody (ANCA) serotypes.@*Methods@#Clinical date of 125 patients diagnosed with AAV by Tianjin Medical University General Hospital from January 1, 2010 to April 30, 2017 were analyzed retrospectively. Clinical manifestations between AAV patients with ILD or those without ILD (NILD) were compared. Patients who were complicated with ILD were divided into myeloperoxidase (MPO)-ANCA positive subset and proteimase (PR3)-ANCA positive subset, and the pulmonary computed tomographic mani-festation was compared among the two subsets. The count data was analyzed by t test, chi-square test/Fisher exact probality. Logistic regression model was applied to analyze the related factors.@*Results@#Of the 125 AAV patients, 86(68.8%) patients were complicated with inter-stitial lung disease, and the mean age of ILD subgroup was higher than that of the NILD subgroup [(66±11) years vs (56±15) years, t=-3.78, P=0.001]. Com-pared with NILD patients, ILD patients had more symptoms (χ2=4.676, P=0.031). The serum levels of carcinoe-mbryonic antigen (t=-1.908, P=0.012), carbohydrate antigen 19-9 (t=-2.286, P=0.025) and carbohydrate antigen 153 (t=-2.857, P=0.007) were higher than the NILD pa-tients. In addition, MPO-ANCA positivesubgroup was more likely to present with pulmonary fibrosis (χ2=7.736, P=0.005), reticular shadow (χ2=9.762, P=0.002) and honeycombing (χ2=4.278, P=0.039) than PR3-ANCA positive subgroup on CT images of all ILD patients. Multivariate Logistic regressive analysis showed that patients who were older than 65 years [OR(95%CI): 3.305(1.280, 8.531), P=0.013], and Birmin-gham vasculitis activity(BVAS) score higher than or equal to 15 [OR(95%CI): 3.249(1.280, 8.247), P=0.013] were risk factors for AAV patients with ILD, while the probability of PR3-ANCA positive patients complicated with ILD was low [OR(95%CI): 0.063(0.005, 0.851), P=0.037].@*Conclusion@#ILD is a very common clinical feature in AAV, especially for elders. The increase of serum CEA, CA199 and CA153 levels may be an early warning sign of interstitial lung disease. Pulmonary HRCT may more likely to present with pulmonary fibrosis in patients with MPO-ANCA positive.
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Objective To investigate the clinical featuresand related factors of anti-neutrophil cytoplasmic antibody associated vasculitis (AVV) with interstitial lung disease (ILD),and to explore the highresolution computed tomography (HRCT) of the chest features between different anti-neutrophil cytoplasmic antibody (ANCA) serotypes.Methods Clinical date of 125 patients diagnosed with AAV by Tianjin Medical University General Hospital from January 1,2010 to April 30,2017 were analyzed retrospectively.Clinical manifestations between AAV patients with ILD or those without ILD (NILD) were compared.Patients who were complicated with ILD were divided into myeloperoxidase (MPO)-ANCA positive subset and proteimase (PR3)-ANCA positive subset,and the pulmonary computed tomographic mani-festation was compared among the two subsets.The count data was analyzed by t test,chi-square test/Fisher exact probality.Logistic regression model was applied to analyze the related factors.Results Of the 125 AAV patients,86 (68.8％) patients were complicated with inter-stitial lung disease,and the mean age of ILD subgroup was higher than that of the NILD subgroup [(66±11) years vs (56±15) years,t=-3.78,P=0.001].Com-pared with NILD patients,ILD patients had more symptoms (x2=4.676,P=0.031).The serum levels of carcinoe-mbryonic antigen (t=-1.908,P=0.012),carbohydrate antigen 19-9 (t=-2.286,P=0.025) and carbohydrate antigen 153 (t=-2.857,P=0.007) were higher than the NILD pa-tients.In addition,MPO-ANCA positivesubgroup was more likely to present with pulmonary fibrosis (x2=7.736,P=0.005),reticular shadow (x2=9.762,P=0.002) and honeycombing (x~=4.278,P=0.039)than PR3-ANCA positive subgroup on CT images of all ILD patients.Multivariate Logistic regressive analysis showed that patients who were older than 65 years [OR(95％CI):3.305(1.280,8.531),P=0.013],and Birmingham vasculitis activity(BVAS) score higher than or equal to 15 [OR(95％CI):3.249(1.280,8.247),P=0.013] were risk factors for AAV patients with ILD,while the probability of PR3-ANCA positive patients complicated with ILD was low [OR (95％CI):0.063 (0.005,0.851),P=0.037].Conclusion ILD is a very common clinical feature in AAV,especially for elders.The increase of serum CEA,CA199 and CA153 levels may be an early warning sign of interstitial lung disease.Pulmonary HRCT may more likely to present with pulmonary fibrosis in patients with MPO-ANCA positive.
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Objective To investigate the change of CD35 expression on neutrophils in the peripheral blood and the relationship between the change and disease activity in patient with myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitis (MPO-AAV).Methods Forty untreated patients with active MPO-AAV(patient group)and forty healthy volunteers (control group) were enrolled into this study,and Bermingham vasculitis activity score (BVAS) for every patient was recorded.Flow cytometry (FCM) was employed to detect the CD35 and MPO expression on the neurtrophil,and enzyme linked immunosorbent assay (ELISA) was taken to test the levels of autoantibody against MPO-Antineutrophil cytoplasmic antibody (MPO-ANCA),fragment a from the activated complement factor B (Ba) and MPO in peripheral blood from both group.All test results were compared between the 2 groups by t test,Non-parametric test,Spearman correlation analysis.In addition,the relations among the laboratory results and the relationship between BVAS and the laboratory results were analyzed respectively.Results Compared with the control group,the expression level,which was represented as mean flourscence indensity (MFI),of CD35 and neutrophil membrane MPO on peripheral blood neutrophils was significantly increased [(2 014±968) vs (1 454±511),t=3.024,P=0.002 and (709±244) vs (580±158),t=2.806,P＜0.01,respectively],and the MPO expression level in neutrophils was significantly lower [(1 525±1 033) vs (3 196±2 126),t=-4.468,P＜0.01].Ba and MPO levels in serum of the patient group was significantly higher than that in the control group [37.89(26.17,63.14) μg/L vs 27.99(18.64,46.52) μg/L,Z=-2.521,P=0.012 and 546.16(450.55,729.96) U/L vs 327.93(279.02,365.10) U/L,Z=7.121,P＜0.01,respectively].In patient group,the expression level of CD35 had a significant positive relationship with peripheral blood neutrophil count (r=0.573,P＜0.01),serum Ba (r=0.433,P=0.005) and BVAS (r=0.368,P=0.020),respectively,whereas,there was a negative correlation between the MPO expressed on the neutrophils and that in the neutrophils (r=-0.458,P=0.003),and a positive relationship between MPO-ANCA and BVAS (r=0.351,P=0.026).Conclusion There is significant increased expression of CD35 on the neutrophil of patient with MPO-AAV,which might protect the neutrophil from destruction by the activated complement alternative pathway,and more neutrophils consequently contribute to the MPO-AAV pathogenesis.Inhibition of CD35 expression might become one of the potential new pathways for the treatment of MPO-AAV.
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Objective To investigate the clinical manifestations,renal pathology and prognosis of antineutrophil cytoplasmic antibody-associated small-vessel vasculitis (AAV) accompanied with renal glomerular IgA deposition.Methods A retrospective analysis was performed at the First Affiliated Hospital of Zhejiang University College of Medicine.Patients diagnosed with AAV associated renal injury by renal biopsy from February 2004 to February 2017 were enrolled.Patients with antiglomerular basement membrane antibody-mediated nephritis,systemic lupus erythematosus nephritis,Henoch Schonlein purpura nephritis,hepatitis B virus associated nephritis and other known etiology were excluded.According to immunofluorescence examination,the patients were divided into IgA deposition group and pauci-immune complex deposition group.The differences in clinical manifestation,pathological features and prognosis were compared between groups.Results A total of 150 AAV cases were included,among which 25 cases were with IgA deposition and 125 cases with pauci-immune complex deposition.The level of serum albumin in IgA deposition group was higher than that in pauci-immune complex deposition group [(35.0±6.2) g/L vs (32.6±5.3) g/L,P=0.049],but the titer of MPO-ANCA was lower [24.8(10.4,71.8) U/ml vs 63.0(21.9,100.0) U/ml,P=0.044] in IgA deposition group.There was no significant difference between two groups in other laboratory indexes and renal pathological findings.The median follow-up time was 15.2 months in IgA deposition group and 8.9 months in pauci immune complex deposition group.During the follow-up there were 8 patients (32.0％) in IgA deposition group and 29 patients (23.2％) in pauci immune complex deposition group on maintaining dialysis;2 patients (8.0％) in IgA deposition group and 7 patients (5.6％) in pauci immune complex deposition group died.There was no significant difference between two groups in patients' outcomes.Conclusions AAV patients with glomerular IgA deposition and AAV patients with typical glomerular immunoglobulin complex deposition are similar as regards clinical appearance and prognosis.
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Objective To investigate the relationship between the incipient serum C-reactive protein (CRP) and clinicopathologic features in anti-neutrophil cytoplasmic antibody associated vasculitis (AAV).Methods Data of 138 consecutive AAV patients were collected.According to their serum CRP levels,patients were divided into group 1 with normal CRP,group 2 with slightly increased CRP and group 3 with severely increased CRP.Clinical features of AAV and histopathologic features of the kidney injury were compared among groups.Results CRP levels increased in 77.53％ AAV patients on admission.Patients in the group of severely increased CRP had the highest levels of BVAS,serum C3,serum ANCA titer,leukocyte counts and the lowest levels of hemoglobin and albumin among the 3 groups (all P ＜ 0.05).The mortality during the stage of therapy was highest in patients with severely increased CRP (P ＜ 0.05).The focal kidney damage was more obvious in patients with severely increased CRP.There was no significant difference in renal prognosis among patients with different CRP levels.Conclusion The levels of incipient serum C-reactive protein of AAV vary in different patients and are positively correlated with patients' inflammation status as well as the disease activity,but are not correlated with the severity of kidney injury.
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Objective To investigate the clinical features of patients with microscopic polyangiitis (MPA) and alveolar hemorrhage (AH),so as to raise the physicians' understanding of this condition.Methods The clinical data of 15 MPA patients combined with AH,who were hospitalized in Peking Union Medical College Hospital from January 2008 to January 2014,were retrospectively analyzed.Results These 15 subjects accounted for 18.1％ of all MPA patients,including 4 males and 11 females with mean age of (59.4 ± 17.9) years old and mean disease duration of 7.5 (1-48) months.Hemoptysis or bloody sputum (14/15) and dyspnea (11/15) were predominant pulmonary manifestations.Hypoxemia appeared in 6 patients (40.0％).An average 8-46 (22.7 ± 4.4) g/L drop of hemoglobin level was seen in all 15 patients.Imaging revealed new bilateral infiltrations or opacities in 13 cases,and bloody bronchoalveolar lavage fluid were found in 6 cases.Compared with MPA patients without AH,the incidence of myalgia [2/15 vs 50.0％ (34/68) ; P =0.009,OR =0.154] and hemoglobin level [(83.2 ± 21.0) g/L vs (102.5 ± 18.7) g/L; P =0.001] were statistically lower in MPA patients with AH.There were no statistical differences in age,sex,interstitial lung disease,renal involvement,peripheral neuropathy and the five factor score between two groups.The proportion of patients who received corticosteroid pulse treatment in MPA with AH group was significantly higher than that of MPA patients without AH [9/15 vs 19.1％ (13/68) ; P =0.003,OR =6.346].After treatment,13 cases achieved remission,while 2 died.Conclusion As an important pulmonary manifestation of MPA,AH could be insidious but fatal.The treatment with steroid,cyclophosphamide and plasma exchange could improve the prognosis.
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ObjectiveTo investigate the outcome and risk factors for kidney involvement by analyzing 64 patients with anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis.MethodsData analyzed including the demographic information,survival status,renal survival status and laboratory parameters such asserum albumin level,serum creatinine level,urinary protein excretion level,hematuria,high sensitivity C-reactive protein(CRP),ANCA titer,and the Birmingham vasculitis activity score (BVAS).Logistic regression analysis,Cox regression analysis and ROC curve were used to evaluate the risk factors of patients with renal involvement and all-event survival.ResultsTotally 64 patients were enrolled [24 females with the average age of (59.9±2.0) years] and followed up for a median of (38±16) months.The morality rate was 14％,and the prevalence of end stage renal disease was 39％.Compared with those who had better outcomes,patients who died or with end stage renal disease had higher serum creatinine level [ (624±246),(245±127 ) μ mol/L,respectively,t=7.17,P＝0.005 ] and erythrocyte sediment rate [ (112±24),(76±48) mm/1 h,respectively,t=3.74,P＜0.01 ],but lower serum albumin level [(294±31 ),(316±42) g/L,respectively,t=-2.27,P=0.01 ] and hemoglobin level [ (79±13),(99±33) g/L,respectively,t=-3.23,P＜0.01 ] at baseline.Logistic regression analysis found that serum creatinine level and erythrocyte sediment rate at baseline were associated with poor outcome and Cox regression analysis further confirmed this result[Scrβ=1.004,95％CI1.002～1.006,P＜0.01; ESR β=l.018,95％CI 1.000～1.037,P=0.046].ROC curve analysis showed that serum creatinine and erythrocyte sediment rate were predictors for AAV patients' prognosis and their AUC were 0.95 and 0.80,the sensitivity of these parameters was both 94％,and the specificity was 93％ and 70％respectively.ConclusionThe intensity of initial treatment should be based on disease severity and activity in order to improve the prognosis of those with ANCA-associated vasculitis with renal involvement.Increased serum creatinine and erythrocyte sediment rate may serve as predictors for poor prognosis in this patient cohort.
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Clinical data were analysed in 19 patients with propylthiouracil (PTU)-induced antineutrophil cytoplasmic antibody associated vasculitis. Among them, 17 patients (89.5%) were diagnosed as Graves′ disease originally. The duration of PTU therapy in 18 patients was more than two years. Multi-organ involvement was common, with 15 patients (78.9%) involved in kidneys and 8 patients (42.1%) involved in lungs. The prevalences of arthralgia, skin rash and fever were also high. All patients withdrew PTU, and some with severe organ involvements received prednisone and immunosuppressant.